Group of chronic blistering diseases characterized histologically by ACANTHOLYSIS and blister formation within the EPIDERMIS.
A desmosomal cadherin that is an autoantigen in the acquired skin disorder PEMPHIGUS VULGARIS.
Separation of the prickle cells of the stratum spinosum of the epidermis, resulting in atrophy of the prickle cell layer. It is seen in diseases such as pemphigus vulgaris (see PEMPHIGUS) and DARIER DISEASE.
A desmosomal cadherin that is an autoantigen in the acquired skin disorder PEMPHIGUS FOLIACEUS.
A group of desmosomal cadherins with cytoplasmic tails that resemble those of classical CADHERINS.
Visible accumulations of fluid within or beneath the epidermis.
Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them.
Skin diseases characterized by local or general distributions of blisters. They are classified according to the site and mode of blister formation. Lesions can appear spontaneously or be precipitated by infection, trauma, or sunlight. Etiologies include immunologic and genetic factors. (From Scientific American Medicine, 1990)
In patients with neoplastic diseases a wide variety of clinical pictures which are indirect and usually remote effects produced by tumor cell metabolites or other products.
A type of junction that attaches one cell to its neighbor. One of a number of differentiated regions which occur, for example, where the cytoplasmic membranes of adjacent epithelial cells are closely apposed. It consists of a circular region of each membrane together with associated intracellular microfilaments and an intercellular material which may include, for example, mucopolysaccharides. (From Glick, Glossary of Biochemistry and Molecular Biology, 1990; Singleton & Sainsbury, Dictionary of Microbiology and Molecular Biology, 2d ed)
A form of fluorescent antibody technique utilizing a fluorochrome conjugated to an antibody, which is added directly to a tissue or cell suspension for the detection of a specific antigen. (Bennington, Saunders Dictionary & Encyclopedia of Laboratory Medicine and Technology, 1984)
A family of related proteins that associate with cytoskeletal elements and junctional complexes at INTERCELLULAR JUNCTIONS. Plakins share a common plakin domain or a plakin repeat domain.
A chronic and relatively benign subepidermal blistering disease usually of the elderly and without histopathologic acantholysis.
An autosomal dominantly inherited skin disorder characterized by recurrent eruptions of vesicles and BULLAE mainly on the neck, axillae, and groin. Mutations in the ATP2C1 gene (encoding the secretory pathway Ca2++/Mn2++ ATPase 1 (SPCA1)) cause this disease. It is clinically and histologically similar to DARIER DISEASE - both have abnormal, unstable DESMOSOMES between KERATINOCYTES and defective CALCIUM-TRANSPORTING ATPASES. It is unrelated to PEMPHIGUS VULGARIS though it closely resembles that disease.
Epidermal cells which synthesize keratin and undergo characteristic changes as they move upward from the basal layers of the epidermis to the cornified (horny) layer of the skin. Successive stages of differentiation of the keratinocytes forming the epidermal layers are basal cell, spinous or prickle cell, and the granular cell.
The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of IgG, for example, IgG1, IgG2A, and IgG2B.
Endogenous tissue constituents that have the ability to interact with AUTOANTIBODIES and cause an immune response.
The external, nonvascular layer of the skin. It is made up, from within outward, of five layers of EPITHELIUM: (1) basal layer (stratum basale epidermidis); (2) spinous layer (stratum spinosum epidermidis); (3) granular layer (stratum granulosum epidermidis); (4) clear layer (stratum lucidum epidermidis); and (5) horny layer (stratum corneum epidermidis).
'Mouth diseases' is a broad term referring to various conditions that cause inflammation, infection, or structural changes in any part of the mouth, including the lips, gums, tongue, palate, cheeks, and teeth, which can lead to symptoms such as pain, discomfort, difficulty in chewing or speaking, and altered aesthetics.
Calcium-dependent cell adhesion proteins. They are important in the formation of ADHERENS JUNCTIONS between cells. Cadherins are classified by their distinct immunological and tissue specificities, either by letters (E- for epithelial, N- for neural, and P- for placental cadherins) or by numbers (cadherin-12 or N-cadherin 2 for brain-cadherin). Cadherins promote cell adhesion via a homophilic mechanism as in the construction of tissues and of the whole animal body.
Desmoplakins are cytoskeletal linker proteins that anchor INTERMEDIATE FILAMENTS to the PLASMA MEMBRANE at DESMOSOMES.
A loss of mucous substance of the mouth showing local excavation of the surface, resulting from the sloughing of inflammatory necrotic tissue. It is the result of a variety of causes, e.g., denture irritation, aphthous stomatitis (STOMATITIS, APHTHOUS); NOMA; necrotizing gingivitis (GINGIVITIS, NECROTIZING ULCERATIVE); TOOTHBRUSHING; and various irritants. (From Jablonski, Dictionary of Dentistry, 1992, p842)
Administration of high doses of pharmaceuticals over short periods of time.
A CALCIUM-dependent adhesion molecule of DESMOSOMES that also plays a role in embryonic STEM CELL proliferation.
A group of desmosomal cadherins with cytoplasmic tails that are divergent from those of classical CADHERINS. Their intracytoplasmic domains bind PLAKOGLOBIN; PLAKOPHILINS; and DESMOPLAKINS.

Explanations for the clinical and microscopic localization of lesions in pemphigus foliaceus and vulgaris. (1/417)

Patients with pemphigus foliaceus (PF) have blisters on skin, but not mucous membranes, whereas patients with pemphigus vulgaris (PV) develop blisters on mucous membranes and/or skin. PF and PV blisters are due to loss of keratinocyte cell-cell adhesion in the superficial and deep epidermis, respectively. PF autoantibodies are directed against desmoglein (Dsg) 1; PV autoantibodies bind Dsg3 or both Dsg3 and Dsg1. In this study, we test the hypothesis that coexpression of Dsg1 and Dsg3 in keratinocytes protects against pathology due to antibody-induced dysfunction of either one alone. Using passive transfer of pemphigus IgG to normal and DSG3(null) neonatal mice, we show that in the areas of epidermis and mucous membrane that coexpress Dsg1 and Dsg3, antibodies against either desmoglein alone do not cause spontaneous blisters, but antibodies against both do. In areas (such as superficial epidermis of normal mice) where Dsg1 without Dsg3 is expressed, anti-Dsg1 antibodies alone can cause blisters. Thus, the anti-desmoglein antibody profiles in pemphigus sera and the normal tissue distributions of Dsg1 and Dsg3 determine the sites of blister formation. These studies suggest that pemphigus autoantibodies inhibit the adhesive function of desmoglein proteins, and demonstrate that either Dsg1 or Dsg3 alone is sufficient to maintain keratinocyte adhesion.  (+info)

Feasibility and safety of a new technique of extracorporeal photochemotherapy: experience of 240 procedures. (2/417)

BACKGROUND AND OBJECTIVE: Extracorporeal photochemotherapy (ECP) is a therapeutic approach based on the biological effects of ultraviolet light (UV) - A and psoralens on mononuclear cells collected by apheresis. Recently, ECP has been under investigation as an alternative treatment for various immune and autoimmune diseases. The aim of this study was to evaluate the safety and feasibility of a new three-step ECP technique, in terms of reproducibility, acceptance, tolerability, and short and long term side effects. DESIGN AND METHODS: Seventeen patients affected by acute or chronic graft-versus-host disease (GvHD), pemphigus vulgaris, or interferon-resistant chronic hepatitis C and one patient being treated for prevention of heart transplant rejection underwent 240 ECP procedures. MNC collection and processing parameters were recorded, biological effects of UV-A/8 methoxy-psoralen (8-MOP) were evaluated, and short and long term side effects were monitored. RESULTS: At a mean follow up of 7 months (range 2-19) 240 ECP had been completed, a mean of 7,136 mL (range 1,998-10,591) of whole blood having beenprocessed per procedure. The mean of total nucleated cells collected per procedure was 6.5x109 (range 0.65-23.8), with a mean MNC percentage of 85% (41. 4-98%) in a mean final volume of 115.5 mL (37-160). No severe side effects were documented and no infectious episodes occurred throughout the course of the treatment. INTERPRETATION AND CONCLUSIONS: The new ECP technique was highly reproducible as regards the collection and each processing step. Short and long term side effects were mild. No increase in infectious episodes was recorded. All patients willingly underwent ECP, demonstrating an excellent tolerability for the procedure even after several courses.  (+info)

The anti-desmoglein 1 autoantibodies in pemphigus vulgaris sera are pathogenic. (3/417)

Pemphigus vulgaris and pemphigus foliaceus are two closely related, but clinically and histologically distinct, autoimmune skin diseases. The autoantigens for pemphigus vulgaris and pemphigus foliaceus are desmoglein 3 and desmoglein 1, respectively. The anti-desmoglein 1 antibodies in pemphigus foliaceus and anti-desmoglein 3 antibodies in pemphigus vulgaris are pathogenic as determined by immunoglobulin G passive transfer animal models. More than 50% of pemphigus vulgaris sera also contain anti-desmoglein 1 autoantibodies; however, the pathogenicity of the anti-desmoglein 1 autoantibodies in pemphigus vulgaris remains unknown. In this study, we used soluble recombinant extracellular domains of desmoglein 1 and desmoglein 3 to obtain affinity-purified anti-desmoglein 1 and anti-desmoglein 3 autoantibodies from pemphigus vulgaris sera and examined the pathogenicity of each fraction separately using the passive transfer mouse model. By immunoprecipitation, the purified anti-desmoglein 1 and anti-desmoglein 3 showed no cross-reactivity. The anti-desmoglein 1 autoantibodies in pemphigus vulgaris induced typical pemphigus foliaceus lesions in neonatal mice, whereas the anti-desmoglein 3 fraction induced pemphigus vulgaris-like lesions. In addition, the pathogenic anti-desmoglein 1 and anti-desmoglein 3 autoantibodies in pemphigus vulgaris had predominant IgG4 subclass specificity. These findings suggest that the anti-desmoglein 1 antibodies in pemphigus vulgaris are pathogenic.  (+info)

Envoplakin and periplakin are the paraneoplastic pemphigus antigens. (4/417)

Paraneoplastic pemphigus (PNP) sera have been reported to immunoprecipitate multiple proteins, including the 250 kDa and 210 kDa proteins believed to correspond to desmoplakins I/II, BP230, and two unidentified proteins of 190 kDa and 170 kDa. We have recently provided evidence that the presence of the 210 kDa and 190 kDa proteins is the most prominent feature of PNP, and have suggested that the major 210 kDa antigen may not correspond to desmoplakin II. Using immunoprecipitation and immunoblotting, we found that some PNP sera identified a doublet protein migrating at 210 kDa, with the larger protein corresponding to desmoplakin II, and the smaller protein corresponding to envoplakin, a recently described precursor of the keratinocyte cornified envelope. In contrast to desmoplakin II, envoplakin was detected by all PNP sera analyzed. Using immunoblotting and immunoprecipitation, we further showed that the 190 kDa PNP antigen is identical to periplakin, another recently identified envelope precursor that can form complexes with envoplakin. Like desmoplakin and BP230, envoplakin and periplakin belong to the plakin family of proteins.  (+info)

Constrictive bronchiolitis obliterans and paraneoplastic pemphigus. (5/417)

Constrictive bronchiolitis obliterans is rare, and the pathogenesis of the disease often remains unknown. This study reports on the case of a 38 yr-old female with constrictive bronchiolitis obliterans and paraneoplastic pemphigus associated with malignant lymphoma. The patient developed progressive obstructive lung disease. The chest radiograph showed almost normal lungs. Paraneoplastic pemphigus is a newly described syndrome in which patients have autoantibodies binding to some epithelia, including in the respiratory tract. The disease develops in association with non-Hodgkin's lymphomas or other malignant neoplasms. The case presented here suggests that constrictive bronchiolitis obliterans associated with paraneoplastic pemphigus may be one of the facets of autoimmune responses in this context.  (+info)

Pemphigus vulgaris and pemphigus foliaceus antibodies are pathogenic in plasminogen activator knockout mice. (6/417)

Previous studies have suggested that urokinase plasminogen activator is required for blister formation in pemphigus vulgaris and pemphigus foliaceus. Other studies, however, have shown that downregulation of plasminogen activator does not inhibit blisters induced by pemphigus immunoglobulin G. To eliminate the possibility that small amounts of urokinase plasminogen activator might be sufficient for blister formation, we passively transferred pemphigus immunoglobulin G to urokinase plasminogen activator knockout neonatal mice. Pemphigus foliaceus and pemphigus vulgaris immunoglobulin G caused gross blisters and acantholysis in the superficial and suprabasal epidermis, respectively, to the same degree in knockout and control mice, demonstrating that urokinase plasminogen activator is not absolutely required for antibody-induced blisters. Some studies have shown elevated tissue-type plasminogen activator in pemphigus lesions. Tissue-type plasminogen activator, however, is not necessary for blister formation, because pemphigus foliaceus and pemphigus vulgaris immunoglobulin G caused blisters to the same degree in tissue-type plasminogen activator knockout and control mice. To rule out that one plasminogen activator might compensate for the other in the knockout mice, we bred urokinase plasminogen activator, tissue-type plasminogen activator double knockouts. After passive transfer of pemphigus foliaceus and pemphigus vulgaris immunoglobulin G these mice blistered to the same degree as the single knockout and control mice, and histology indicated blisters at the expected level of the epidermis. These data definitively demonstrate that plasminogen activator is not necessary for pemphigus immunoglobulin G to induce acantholysis in the neonatal mouse model of pemphigus.  (+info)

Common human leukocyte antigen alleles in pemphigus vulgaris and pemphigus foliaceus Italian patients. (7/417)

Pemphigus refers to a group of autoimmune blistering skin diseases, mainly identified as pemphigus vulgaris and pemphigus foliaceus, both characterized by the presence of autoantibodies against keratinocyte adhesion molecules, leading to loss of cell-cell adhesion with consequent blister formation. Pemphigus vulgaris is reported to be associated with human leukocyte antigen DR4 and/or DR6 whereas no data are available on pemphigus foliaceus, except for the endemic Brazilian form (fogo selvagem), which is reported to be associated with DR1 and DR4. We here report human leukocyte antigen molecular typing on a total of 87 patients, 61 with pemphigus vulgaris and 26 with pemphigus foliaceus, versus 128 healthy matched controls. Generic typing showed an increase of DRB1*04 and DRB1*14 and a decrease of DRB1*07 in both pemphigus vulgaris and pemphigus foliaceus patients. Molecular subtyping of DR4+ and DR14+ subjects showed a highly significant association between the DRB1*1401 and both pemphigus vulgaris (p < 0.0001) and pemphigus foliaceus patients (p < 0.0001) together with a significant increase of the linked DQB1*0503 (pemphigus vulgaris p < 0.0001; pemphigus foliaceus p < 0.0001). Moreover, whereas the association between DRB1*0402 and pemphigus vulgaris (p < 0.0001) has been confirmed, no significant association between a specific allele of the DR4 group and pemphigus foliaceus, has been found. Therefore, at least in Italian patients, pemphigus vulgaris and pemphigus foliaceus share DRB1*1401 and DQB1*0503, as susceptible human leukocyte antigen alleles, whereas DRB1*0402 is only found associated with pemphigus vulgaris. The observation that both diseases, pemphigus vulgaris and pemphigus foliaceus, carry the same susceptible human leukocyte antigen alleles has been interpreted as a common genetic background predisposing to pemphigus as, like in other autoimmune disorders, it is not sufficient to explain the onset of the disease on the basis of the sole aforementioned alleles. Other linked genes and/or environmental factors should play a facilitating role in the outbreak of pemphigus, either pemphigus vulgaris or pemphigus foliaceus.  (+info)

The role of antibody to human beta4 integrin in conjunctival basement membrane separation: possible in vitro model for ocular cicatricial pemphigoid. (8/417)

PURPOSE: To demonstrate the specific binding of autoantibodies present in the sera of patients with ocular cicatricial pemphigoid (OCP) to human beta4 integrin present in the normal human conjunctiva (NHC) and to study the role of OCP autoantibodies and antibody to human beta4 integrin in the pathogenesis of subepithelial lesion formation in OCP. METHODS: Indirect immunofluorescence assay and in vitro organ culture method using NHC were used. Sera and IgG fractions from 10 patients with OCP; immunoaffinity-purified OCP autoantibody; antibodies to human beta4, beta1, alpha6, and alpha5 integrins; and sera from patients with pemphigus vulgaris, bullous pemphigoid (BP), and chronic atopic and chronic ocular rosacea cicatrizing conjunctivitis; and normal human serum (NHS) were used. RESULTS: Nine of 10 OCP sera or IgG fractions, immunoaffinity-purified OCP autoantibody, antibodies to human beta4 and alpha6 integrins, and sera from patients with BP showed homogenous, smooth linear binding along the basement membrane zone (BMZ) of the NHC. NHS, antibodies to other integrins, and sera from patients with chronic cicatrizing conjunctivitis from other causes showed no such binding. When NHC was first absorbed with OCP sera and then reacted with anti-beta4 antibodies or vice versa, the intensity of the BMZ binding was dramatically reduced or completely eliminated, indicating that there were autoantibodies in OCP sera specific for the beta4 integrin. BMZ separation developed 48 to 72 hours after addition of total OCP sera, IgG fractions from OCP sera, immunoaffinity-purified autoantibodies from sera of patients with OCP, or anti-beta4 antibodies to the NHC cultures, but not after addition of normal control sera, sera from patients with chronic cicatrizing conjunctivitis from causes other than OCP, or sera from patients with OCP in clinical remission. CONCLUSION: Circulating anti-beta4 integrin antibody may have an important role in the pathogenesis of OCP.  (+info)

Pemphigus is a group of rare, autoimmune blistering diseases that affect the skin and mucous membranes. In these conditions, the immune system mistakenly produces antibodies against desmoglein proteins, which are crucial for maintaining cell-to-cell adhesion in the epidermis (outermost layer of the skin). This results in the loss of keratinocyte cohesion and formation of flaccid blisters filled with serous fluid.

There are several types of pemphigus, including:

1. Pemphigus vulgaris - The most common form, primarily affecting middle-aged to older adults, with widespread erosions and flaccid blisters on the skin and mucous membranes (e.g., mouth, nose, genitals).
2. Pemphigus foliaceus - A more superficial form, mainly involving the skin, causing crusted erosions and scaly lesions without mucosal involvement. It is more prevalent in older individuals and in certain geographical regions like the Middle East.
3. Paraneoplastic pemphigus - A rare type associated with underlying neoplasms (cancers), such as lymphomas or carcinomas, characterized by severe widespread blistering of both skin and mucous membranes, along with antibodies against additional antigens besides desmogleins.
4. IgA pemphigus - A less common form characterized by localized or generalized erosions and blisters, with IgA autoantibodies targeting the basement membrane zone.

Treatment for pemphigus typically involves high-dose systemic corticosteroids, often in combination with immunosuppressive agents (e.g., azathioprine, mycophenolate mofetil, rituximab) to control the disease activity and prevent complications. Regular follow-ups with dermatologists and oral specialists are essential for monitoring treatment response and managing potential side effects.

Desmoglein 3 is a type of desmoglein protein that is primarily found in the upper layers of the epidermis, specifically in the desmosomes of the skin. Desmogleins are part of the cadherin family of cell adhesion molecules and play a crucial role in maintaining the structural integrity and cohesion of tissues, particularly in areas subjected to mechanical stress.

Desmoglein 3 is essential for the formation and maintenance of desmosomal junctions in stratified squamous epithelia, such as the skin and mucous membranes. It is involved in cell-to-cell adhesion by forming calcium-dependent homophilic interactions with other Desmoglein 3 molecules on adjacent cells.

Mutations in the gene encoding Desmoglein 3 have been associated with several skin disorders, including pemphigus vulgaris, a severe autoimmune blistering disease that affects the mucous membranes and skin. In pemphigus vulgaris, autoantibodies target Desmoglein 3 (and sometimes Desmoglein 1) molecules, leading to loss of cell-to-cell adhesion and formation of blisters and erosions.

Acantholysis is a medical term that refers to the separation of the cells in the upper layer of the skin (the epidermis), specifically between the pickle cell layer (stratum spinosum) and the granular cell layer (stratum granulosum). This separation results in the formation of distinct, round, or oval cells called acantholytic cells, which are typically seen in certain skin conditions.

Acantholysis is a characteristic feature of several skin disorders, including:

1. Pemphigus vulgaris: A rare autoimmune blistering disorder where the immune system produces antibodies against desmoglein-1 and -3 proteins, leading to acantholysis and formation of flaccid blisters.
2. Pemphigus foliaceus: Another autoimmune blistering disorder that specifically targets desmoglein-1 protein, causing superficial blisters and erosions on the skin.
3. Hailey-Hailey disease (familial benign chronic pemphigus): An autosomal dominant genetic disorder affecting ATP2C1 gene, leading to defective calcium transport and abnormal keratinocyte adhesion, resulting in acantholysis and recurrent skin eruptions.
4. Darier's disease (keratosis follicularis): An autosomal dominant genetic disorder affecting ATP2A2 gene, causing dysfunction of calcium transport and abnormal keratinocyte adhesion, resulting in acantholysis and characteristic papular or keratotic skin lesions.
5. Grover's disease (transient acantholytic dermatosis): An acquired skin disorder of unknown cause, characterized by the development of pruritic, red, and scaly papules and vesicles due to acantholysis.

The presence of acantholysis in these conditions can be confirmed through histopathological examination of skin biopsies.

Desmoglein 1 is a type of desmosomal cadherin, which is a transmembrane protein involved in cell-to-cell adhesion. It is primarily expressed in the upper layers of the epidermis and plays a crucial role in maintaining the integrity and stability of the skin. Desmoglein 1 forms desmosomes, specialized intercellular junctions that connect adjacent keratinocytes and help to resist shearing forces.

Desmoglein 1 is also a target for autoantibodies in certain blistering diseases, such as pemphigus foliaceus, where the binding of these antibodies to desmoglein 1 results in the loss of cell-to-cell adhesion and formation of skin blisters.

Desmogleins are a group of proteins that are part of the desmosomes, which are structures that help to strengthen and maintain the integrity of epithelial tissues. Desmogleins play a crucial role in cell-to-cell adhesion by forming intercellular junctions known as desmoglein adherens junctions. These junctions help to anchor intermediate filaments, such as keratin, to the plasma membrane and provide structural support to epithelial cells.

There are four main types of desmogleins (Dsg1-4), each with distinct expression patterns in different tissues. For example, Dsg1 is primarily expressed in the upper layers of the epidermis, while Dsg3 is found in the lower layers and in mucous membranes. Mutations in desmoglein genes have been associated with several skin disorders, including pemphigus vulgaris and pemphigus foliaceus, which are autoimmune blistering diseases characterized by the loss of cell-to-cell adhesion in the epidermis.

A blister is a small fluid-filled bubble that forms on the skin due to friction, burns, or contact with certain chemicals or irritants. Blisters are typically filled with a clear fluid called serum, which is a component of blood. They can also be filled with blood (known as blood blisters) if the blister is caused by a more severe injury.

Blisters act as a natural protective barrier for the underlying skin and tissues, preventing infection and promoting healing. It's generally recommended to leave blisters intact and avoid breaking them, as doing so can increase the risk of infection and delay healing. If a blister is particularly large or painful, medical attention may be necessary to prevent complications.

Autoantibodies are defined as antibodies that are produced by the immune system and target the body's own cells, tissues, or organs. These antibodies mistakenly identify certain proteins or molecules in the body as foreign invaders and attack them, leading to an autoimmune response. Autoantibodies can be found in various autoimmune diseases such as rheumatoid arthritis, lupus, and thyroiditis. The presence of autoantibodies can also be used as a diagnostic marker for certain conditions.

Vesiculobullous skin diseases are a group of disorders characterized by the formation of blisters (vesicles) and bullae (larger blisters) on the skin. These blisters form when there is a separation between the epidermis (outer layer of the skin) and the dermis (layer beneath the epidermis) due to damage in the area where they join, known as the dermo-epidermal junction.

There are several types of vesiculobullous diseases, each with its own specific causes and symptoms. Some of the most common types include:

1. Pemphigus vulgaris: an autoimmune disorder where the immune system mistakenly attacks proteins that help to hold the skin together, causing blisters to form.
2. Bullous pemphigoid: another autoimmune disorder, but in this case, the immune system attacks a different set of proteins, leading to large blisters and inflammation.
3. Dermatitis herpetiformis: a skin condition associated with celiac disease, where gluten ingestion triggers an immune response that leads to the formation of itchy blisters.
4. Pemphigoid gestationis: a rare autoimmune disorder that occurs during pregnancy and causes blisters on the abdomen and other parts of the body.
5. Epidermolysis bullosa: a group of inherited disorders where there is a fragile skin structure, leading to blistering and wound formation after minor trauma or friction.

Treatment for vesiculobullous diseases depends on the specific diagnosis and may include topical or systemic medications, such as corticosteroids, immunosuppressants, or antibiotics, as well as wound care and prevention of infection.

Paraneoplastic syndromes refer to a group of rare disorders that are caused by an abnormal immune system response to a cancerous (malignant) tumor. These syndromes are characterized by symptoms or signs that do not result directly from the growth of the tumor itself, but rather from substances produced by the tumor or the body's immune system in response to the tumor.

Paraneoplastic syndromes can affect various organs and systems in the body, including the nervous system, endocrine system, skin, and joints. Examples of paraneoplastic syndromes include Lambert-Eaton myasthenic syndrome (LEMS), which affects nerve function and causes muscle weakness; cerebellar degeneration, which can cause difficulty with coordination and balance; and dermatomyositis, which is an inflammatory condition that affects the skin and muscles.

Paraneoplastic syndromes can occur in association with a variety of different types of cancer, including lung cancer, breast cancer, ovarian cancer, and lymphoma. Treatment typically involves addressing the underlying cancer, as well as managing the symptoms of the paraneoplastic syndrome.

Desmosomes are specialized intercellular junctions that provide strong adhesion between adjacent epithelial cells and help maintain the structural integrity and stability of tissues. They are composed of several proteins, including desmoplakin, plakoglobin, and cadherins, which form complex structures that anchor intermediate filaments (such as keratin) to the cell membrane. This creates a network of interconnected cells that can withstand mechanical stresses. Desmosomes are particularly abundant in tissues subjected to high levels of tension, such as the skin and heart.

The Fluorescent Antibody Technique (FAT), Direct is a type of immunofluorescence assay used in laboratory diagnostic tests. It is a method for identifying and locating specific antigens in cells or tissues by using fluorescent-labeled antibodies that directly bind to the target antigen.

In this technique, a sample (such as a tissue section or cell smear) is prepared and then treated with a fluorescently labeled primary antibody that specifically binds to the antigen of interest. After washing away unbound antibodies, the sample is examined under a fluorescence microscope. If the antigen is present in the sample, it will be visible as distinct areas of fluorescence, allowing for the direct visualization and localization of the antigen within the cells or tissues.

Direct FAT is commonly used in diagnostic laboratories to identify and diagnose various infectious diseases, including bacterial, viral, and fungal infections. It can also be used to detect specific proteins or antigens in research and clinical settings.

Plakins are a family of proteins that play important roles in maintaining the structure and function of various types of cells, particularly in epithelial tissues. They are large, multidomain proteins that interact with several other cellular components, including the cytoskeleton, cell adhesion molecules, and extracellular matrix proteins.

The name "plakin" comes from the Greek word "plax," which means "plate" or "plaque." This reflects the fact that these proteins help to form and maintain cell-cell and cell-matrix junctions, which are often referred to as "plaques" due to their plate-like appearance.

There are several different types of plakins, including:

1. BP230 (also known as BPAG1-e): This plakin is a component of hemidesmosomes, which are structures that help to anchor epithelial cells to the underlying basement membrane.
2. Plectin: This plakin is a large protein that interacts with several different components of the cytoskeleton, including intermediate filaments, microtubules, and actin filaments. It is found in many different types of cells, including epithelial cells, muscle cells, and neurons.
3. Desmoplakin: This plakin is a component of desmosomes, which are structures that help to anchor adjacent epithelial cells together.
4. Periplakin: This plakin is found in the upper layers of the skin, where it helps to form and maintain cell-cell junctions called corneodesmosomes.
5. Microtubule actin crosslinking factor 1 (MACF1): This plakin interacts with both microtubules and actin filaments, and is involved in regulating the organization and dynamics of these cytoskeletal components.

Mutations in genes encoding plakins have been associated with a variety of human diseases, including epidermolysis bullosa, a group of inherited skin disorders characterized by fragile skin and blistering.

According to the American Academy of Ophthalmology and the National Organization for Rare Disorders, bullous pemphigoid is an autoimmune blistering disorder characterized by the formation of large, fluid-filled blisters (bullae) on the skin and mucous membranes. This condition primarily affects older adults, with most cases occurring in individuals over 60 years of age.

In bullous pemphigoid, the immune system mistakenly produces antibodies against proteins called BP230 and BP180, which are found in the basement membrane zone – a layer that separates the epidermis (outer skin layer) from the dermis (inner skin layer). This autoimmune response leads to the formation of blisters, causing significant discomfort and potential complications if left untreated.

The symptoms of bullous pemphigoid typically include:

1. Large, fluid-filled blisters on the skin, often appearing on the trunk, arms, or legs. These blisters may be itchy or painful.
2. Blisters that rupture easily, leading to raw, open sores.
3. Mucous membrane involvement, such as blisters in the mouth, nose, eyes, or genital area.
4. Skin redness and irritation.
5. Fluid-filled bumps (papules) or pus-filled bumps (pustules).
6. Scarring and skin discoloration after blisters heal.

Treatment for bullous pemphigoid usually involves a combination of medications to control the immune response, reduce inflammation, and promote healing. These may include corticosteroids, immunosuppressants, or other targeted therapies. In some cases, antibiotics may also be prescribed to help manage secondary infections that can occur due to blister formation.

It is essential to consult with a healthcare professional for an accurate diagnosis and treatment plan if you suspect you have bullous pemphigoid or are experiencing related symptoms.

Benign familial pemphigus is a rare, autosomal dominant blistering disorder that primarily affects the mucous membranes. It is characterized by the presence of flaccid blisters and erosions on the skin and mucous membranes. The lesions are usually painless and heal without scarring.

The condition is caused by mutations in the desmoglein-1 (DSG1) gene, which provides instructions for making a protein called desmoglein 1. This protein is a component of desmosomes, which are structures that help bind cells together. Mutations in the DSG1 gene lead to the production of an abnormal desmoglein 1 protein, which disrupts the formation of desmosomes and causes the cells in the epidermis to separate from each other, resulting in blister formation.

Benign familial pemphigus is typically a milder form of pemphigus and has a good prognosis. Treatment usually involves the use of topical corticosteroids to reduce inflammation and promote healing of the lesions. In severe cases, systemic corticosteroids or other immunosuppressive medications may be necessary.

Keratinocytes are the predominant type of cells found in the epidermis, which is the outermost layer of the skin. These cells are responsible for producing keratin, a tough protein that provides structural support and protection to the skin. Keratinocytes undergo constant turnover, with new cells produced in the basal layer of the epidermis and older cells moving upward and eventually becoming flattened and filled with keratin as they reach the surface of the skin, where they are then shed. They also play a role in the immune response and can release cytokines and other signaling molecules to help protect the body from infection and injury.

Immunoglobulin G (IgG) is a type of antibody, which is a protective protein produced by the immune system in response to foreign substances like bacteria or viruses. IgG is the most abundant type of antibody in human blood, making up about 75-80% of all antibodies. It is found in all body fluids and plays a crucial role in fighting infections caused by bacteria, viruses, and toxins.

IgG has several important functions:

1. Neutralization: IgG can bind to the surface of bacteria or viruses, preventing them from attaching to and infecting human cells.
2. Opsonization: IgG coats the surface of pathogens, making them more recognizable and easier for immune cells like neutrophils and macrophages to phagocytose (engulf and destroy) them.
3. Complement activation: IgG can activate the complement system, a group of proteins that work together to help eliminate pathogens from the body. Activation of the complement system leads to the formation of the membrane attack complex, which creates holes in the cell membranes of bacteria, leading to their lysis (destruction).
4. Antibody-dependent cellular cytotoxicity (ADCC): IgG can bind to immune cells like natural killer (NK) cells and trigger them to release substances that cause target cells (such as virus-infected or cancerous cells) to undergo apoptosis (programmed cell death).
5. Immune complex formation: IgG can form immune complexes with antigens, which can then be removed from the body through various mechanisms, such as phagocytosis by immune cells or excretion in urine.

IgG is a critical component of adaptive immunity and provides long-lasting protection against reinfection with many pathogens. It has four subclasses (IgG1, IgG2, IgG3, and IgG4) that differ in their structure, function, and distribution in the body.

Autoantigens are substances that are typically found in an individual's own body, but can stimulate an immune response because they are recognized as foreign by the body's own immune system. In autoimmune diseases, the immune system mistakenly attacks and damages healthy tissues and organs because it recognizes some of their components as autoantigens. These autoantigens can be proteins, DNA, or other molecules that are normally present in the body but have become altered or exposed due to various factors such as infection, genetics, or environmental triggers. The immune system then produces antibodies and activates immune cells to attack these autoantigens, leading to tissue damage and inflammation.

The epidermis is the outermost layer of the skin, composed mainly of stratified squamous epithelium. It forms a protective barrier that prevents water loss and inhibits the entry of microorganisms. The epidermis contains no blood vessels, and its cells are nourished by diffusion from the underlying dermis. The bottom-most layer of the epidermis, called the stratum basale, is responsible for generating new skin cells that eventually move up to replace dead cells on the surface. This process of cell turnover takes about 28 days in adults.

The most superficial part of the epidermis consists of dead cells called squames, which are constantly shed and replaced. The exact rate at which this happens varies depending on location; for example, it's faster on the palms and soles than elsewhere. Melanocytes, the pigment-producing cells, are also located in the epidermis, specifically within the stratum basale layer.

In summary, the epidermis is a vital part of our integumentary system, providing not only physical protection but also playing a crucial role in immunity and sensory perception through touch receptors called Pacinian corpuscles.

Mouth diseases refer to a variety of conditions that affect the oral cavity, including the lips, gums, teeth, tongue, palate, and lining of the mouth. These diseases can be caused by bacteria, viruses, fungi, or other organisms. They can also result from injuries, chronic illnesses, or genetic factors.

Some common examples of mouth diseases include dental caries (cavities), periodontal disease (gum disease), oral herpes, candidiasis (thrush), lichen planus, and oral cancer. Symptoms may include pain, swelling, redness, bleeding, bad breath, difficulty swallowing or speaking, and changes in the appearance of the mouth or teeth. Treatment depends on the specific diagnosis and may involve medications, dental procedures, or lifestyle changes.

Cadherins are a type of cell adhesion molecule that play a crucial role in the development and maintenance of intercellular junctions. They are transmembrane proteins that mediate calcium-dependent homophilic binding between adjacent cells, meaning that they bind to identical cadherin molecules on neighboring cells.

There are several types of cadherins, including classical cadherins, desmosomal cadherins, and protocadherins, each with distinct functions and localization in tissues. Classical cadherins, also known as type I cadherins, are the most well-studied and are essential for the formation of adherens junctions, which help to maintain cell-to-cell contact and tissue architecture.

Desmosomal cadherins, on the other hand, are critical for the formation and maintenance of desmosomes, which are specialized intercellular junctions that provide mechanical strength and stability to tissues. Protocadherins are a diverse family of cadherin-related proteins that have been implicated in various developmental processes, including neuronal connectivity and tissue patterning.

Mutations in cadherin genes have been associated with several human diseases, including cancer, neurological disorders, and heart defects. Therefore, understanding the structure, function, and regulation of cadherins is essential for elucidating their roles in health and disease.

Desmoplakins are important proteins that play a crucial role in the structural integrity and function of certain types of cell-to-cell junctions called desmosomes. Desmosomes are specialized structures that connect adjacent cells in tissues that undergo significant mechanical stress, such as the skin, heart, and gut.

Desmoplakins are large proteins that are composed of several domains, including a plakin domain, which interacts with other desmosomal components, and a spectrin-like repeat domain, which binds to intermediate filaments. By linking desmosomes to the intermediate filament network, desmoplakins help to provide mechanical strength and stability to tissues.

Mutations in the genes that encode desmoplakins have been associated with several human genetic disorders, including arrhythmogenic right ventricular cardiomyopathy (ARVC), a heart condition characterized by abnormal heart rhythms and structural changes in the heart muscle, and epidermolysis bullosa simplex (EBS), a skin disorder characterized by blistering and fragility of the skin.

An oral ulcer is a defect or break in the continuity of the epithelium, the tissue that lines the inner surface of the mouth, leading to an inflamed, painful, and sometimes bleeding lesion. They can be classified as primary (e.g., aphthous ulcers, traumatic ulcers) or secondary (e.g., those caused by infections, underlying systemic conditions, or reactions to medications). Oral ulcers may cause discomfort, impacting speech and food consumption, and their presence might indicate an underlying medical issue that requires further evaluation.

Pulse therapy, in the context of drug treatment, refers to a therapeutic regimen where a medication is administered in large doses for a short period of time, followed by a break or "drug-free" interval before the next dose. This cycle is then repeated at regular intervals. The goal of pulse therapy is to achieve high concentrations of the drug in the body to maximize its therapeutic effect while minimizing overall exposure and potential side effects.

This approach is often used for drugs that have a long half-life or slow clearance, as it allows for periodic "washing out" of the drug from the body. Pulse therapy can also help reduce the risk of developing drug resistance in certain conditions like rheumatoid arthritis and tuberculosis. Common examples include pulse methotrexate for rheumatoid arthritis and intermittent preventive treatment with anti-malarial drugs.

It is important to note that the use of pulse therapy should be based on a thorough understanding of the drug's pharmacokinetics, therapeutic index, and potential adverse effects. Close monitoring of patients undergoing pulse therapy is essential to ensure safety and efficacy.

Desmoglein 2 is a type of desmoglein protein that is primarily found in the desmosomes of epithelial cells. Desmosomes are specialized structures that help to anchor intermediate filaments to the cell membrane and provide strength and stability to tissues that undergo mechanical stress, such as the skin and heart.

Desmoglein 2 plays a critical role in maintaining cell-cell adhesion by forming intercellular junctions called desmosomal cadherins. These junctions help to hold adjacent cells together and contribute to the integrity of epithelial tissues. Mutations in the gene that encodes Desmoglein 2 have been associated with several skin disorders, including pemphigus vulgaris, a blistering autoimmune disease that affects mucous membranes and the skin. In this condition, antibodies target Desmoglein 2, leading to loss of cell-cell adhesion and formation of blisters.

Desmocollins are a type of cadherin, which is a transmembrane protein involved in cell-cell adhesion. Specifically, desmocollins are found in the desmosomes, which are specialized structures that help to mechanically connect adjacent epithelial cells. There are three main isoforms of desmocollin (Desmocollin-1, -2, and -3) that are encoded by different genes. Mutations in the genes encoding desmocollins have been associated with several skin blistering disorders, including certain forms of epidermolysis bullosa.

... vulgaris Pemphigus vegetans Pemphigus vegetans of Hallopeau Pemphigus vegetans of Neumann Pemphigus foliaceus, of ... pemphigus vulgaris (PV) and pemphigus foliaceus (PF). However, several other disorders such as IgA pemphigus, IgE pemphigus, ... the new variant endemic pemphigus Foliaeus and Tunisian endemic pemphigus foliaceus Paraneoplastic pemphigus IgA pemphigus, of ... The several types of pemphigus (pemphigus vulgaris, pemphigus foliaceus, intraepidermal neutrophilic IgA dermatosis, and ...
... is a cutaneous condition, a clinical variant of pemphigus that combines the clinical features of ... Pemphigus Herpetiformis is an IGg mediated autoantibodies that affect the epidermal layer of the skin. Adult linear IgA disease ... ISBN 978-1-4160-2999-1. Sandhu, Neil (2019-01-09). "Pemphigus Herpetiformis: Background, Pathophysiology, Epidemiology". Neil ... dermatitis herpetiformis with the immunopathologic features of pemphigus. ...
... is a rare chronic blistering skin disease and the most common form of pemphigus. Pemphigus was derived from ... "Pemphigus". www.pemphigus.org. Retrieved 2019-12-03. "Pemphigus Vulgaris , Causes, Symptoms and Treatment". patient.info. 2 May ... Beigi PK (2018). "History of Pemphigus Vulgaris". A Clinician's Guide to Pemphigus Vulgaris. Springer, Cham. p. 13. doi:10.1007 ... Nevertheless, in 1998, pemphigus vulgaris was the fourth most common cause of death due to a skin disorder. It is thus still ...
... is a species of gall-forming aphid. It creates galls on the leaves of Populus fremontii. The species is ... Aoki, Shigeyuki; Moran, Nancy A. (1994). "Pemphigus Obesinymphae, a New American Aphid Species with Defenders and Swollen ...
... also known as poplar petiole gall aphid or cabbage root aphid, induces galls on the leaves of ... Galls are induced by female Pemphigus populitransversus on the base of leaves in black cottonwood, narrowleaf cottonwood, ...
... ~workup at eMedicine Anhalt, Grant J.; Kim, SooChan; Stanley, John R.; Korman, Neil J.; Jabs, Douglas A ... Paraneoplastic pemphigus (PNP) is an autoimmune disorder stemming from an underlying tumor. It is hypothesized that antigens ... The article warned clinicians to be alert to the possibility that paraneoplastic pemphigus in lymphomas not of B-cell lineage. ... PNP is most commonly mistaken for pemphigus vulgaris, due to the extreme similarities of the lesions that develop. However, the ...
... is simply a localized form of pemphigus foliaceus with features of lupus erythematosus. Pemphigus List ...
Favret, C. (2018). "species Pemphigus (Pemphigus) betae". Aphid Species File (Version 5.0/5.0). Retrieved 8 May 2018. Hutchison ... Pemphigus betae, also known as the sugarbeet root aphid, is a species of gall-forming aphid that forms galls specifically on ... "Sugarbeet Root Aphid Pemphigus populivenae Fitch". Identification Keys for Insect Pests in Pacific Northwest Field Crops. ... There is intense competition between Pemphigus stem mothers over leaf choice - galls formed on larger leaves have higher stem ...
... is a subtype of pemphigus with two distinct forms: Subcorneal pustular dermatosis (also known as Sneddon- ... Pemphigus List of cutaneous conditions List of conditions caused by problems with junctional proteins List of ... 203 This is distinct from and not to be confused with subcorneal pustular dermatosis type of IgA pemphigus. Sneddon's syndrome ...
... is included in the genus Pemphigus. The aphid soldier exhibits its first distinguishing physical traits ... Pemphigus spyrothecae, or the poplar spiral gall aphid, is a social insect which exhibits apparent altruistic behaviors. The ... Pemphigus spyrothecae is in the superfamily Aphidoidea, in the hymopterous division of the order Hemiptera, which consists of ... "Pemphigus spyrothecae". {{cite web}}: ,last= has generic name (help) Floate, Kevin D. (2010). Gall-Inducing Aphids and Mites ...
1863 Pemphigus cylindricus Zhang & Guangxue, 1981 Pemphigus diani Ferrari, 1872 Pemphigus dorocola Pemphigus eastopi Pemphigus ... 1964 Pemphigus groenlandicus Rübsaamen, 1898 Pemphigus hydrophilus Pemphigus immunis Buckton, 1896 Pemphigus indicus Pemphigus ... 1929 Pemphigus saccosus Pemphigus saliciradicis (Börner, 1950) Pemphigus salicis Pemphigus similis Börner, 1950 Pemphigus ... Species: Pemphigus andropogiae Shinji, 1924 Pemphigus betae Doane, 1900 Pemphigus birimatus Pemphigus borealis Tullgren, 1909 ...
... of pemphigus cases and is a relatively benign variant of pemphigus vulgaris. Two forms are recognized: Pemphigus vegetans of ... Pemphigus vegetans of Hallopeau is a disease of localized pemphigus vulgaris. : 568 It is named for François Henri Hallopeau. ... Pemphigus vegetans is a localized form of pemphigus vulgaris. in which there is a localized vegetating papillomatous response. ... 568 This type is more common and characterized by early lesions similar to Pemphigus Vulgaris with large bullae and erosive ...
... is an autoimmune (blistering disease) of the skin. Pemphigus foliaceus causes a characteristic inflammatory ... and testing either a blood sample or a skin sample for the antibodies that cause pemphigus.[citation needed] Pemphigus is ... In people with pemphigus, however, the immune system mistakenly attacks the cells in the epidermis, or top layer of the skin, ... Pemphigus foliaceus is diagnosed base on history, biopsy of the affected skin, ...
Pemphigus vegetans of Hallopeau represents a mild form of pemphigus vegetans. Pemphigus vegetans of Neumann represents a severe ... form of pemphigus vegetans. List of target antigens in pemphigoid List of conditions caused by problems with junctional ... a list of antigens in the skin that may become targets of circulating auto-antibodies leading to the various types of pemphigus ...
For example, paraneoplastic pemphigus often included infection as a major cause of death. Paraneoplastic pemphigus is one of ... Kasperkiewicz M, Ellebrecht CT, Takahashi H, Yamagami J, Zillikens D, Payne AS, Amagai M (May 2017). "Pemphigus". Nature ... October 2012). "Prognostic factors of paraneoplastic pemphigus". Archives of Dermatology. 148 (10): 1165-1172. doi:10.1001/ ...
Pemphigus foliaceus is an autoimmune skin disorder, believed to be genetic. Systemic lupus erythematosus, or lupus, is a ... "Pemphigus". The Akita Association (UK). Archived from the original (website) on 3 March 2016. Retrieved 22 April 2011. " ... Kuhl, K. A.; Shofer, F. S.; Goldschmidt, M. H. (1994). "Comparative Histopathology of Pemphigus Foliaceus and Superficial ...
Pemphigus • Peninsula College of Medicine and Dentistry • Pennsylvania College of Dental Surgery • Pepsodent • Periapical ...
List of keratins expressed in the human integumentary system List of target antigens in pemphigus List of immunofluorescence ... "Pemphigus foliaceus". Dermatology Online Journal. 20 (12).{{cite journal}}: CS1 maint: multiple names: authors list (link) ... Beigi, Pooya Khan Mohammad (2018). "Background". A Clinician's Guide to Pemphigus Vulgaris. Springer, Cham. pp. 3-10. doi: ...
It is also associated with pemphigus vulgaris and pemphigus foliaceus. It is useful in differentiating between the diagnosis of ... Corwin, J (2016). "Pemphigus vulgaris. In: Ferri FF, editor". Ferri's Clinical Advisor: 945-6. (CS1 errors: missing periodical ... "eMedicine - Pemphigus Foliaceus : Article by Robert A Schwartz". 2018-04-03. {{cite journal}}: Cite journal requires ,journal ... Materiali K.uchenigu o pemphigus foliaceus [doctoral thesis]. Kiev. 1896. "MedilinePlus: Nikolsky's sign". Asz J, Asz D, ...
An autoimmune disease called pemphigus is also caused by loss of cell adhesion, as it results from autoantibodies targeting a ... Tamgadge, Sandhya; Bhatt, DaivatM; Pereira, Treville; Tamgadge, Avinash; Bhalerao, Sudhir (2011). "Pemphigus vulgaris". ...
List of target antigens in pemphigus List of conditions caused by problems with junctional proteins GRCh38: Ensembl release 89 ... Autoantibodies to DSP are a hallmark of the autoimmune disease paraneoplastic pemphigus. Decreased desmoplakin expression has ... "Paraneoplastic pemphigus. An autoimmune mucocutaneous disease associated with neoplasia". The New England Journal of Medicine. ... Carvajal syndrome and paraneoplastic pemphigus. Desmoplakin exists as two predominant isoforms; the first, known as "DPII", has ...
ISBN 978-0-8121-0641-1. "Pemphigus: A Historical Perspective". International Pemphigus Pemphigoid Foundation (IPPF). 31 January ... In 1953 with Eric Waddington, Rook wrote on bullous (blistering) skin diseases and believed that pemphigus was distinct from ... ISBN 9780521405294 Rook, A. J.; Whimster, I. W. (1950). "The Histological Diagnosis of Pemphigus". British Journal of ... Rook, Arthur; Waddington, Eric (1953). "Pemphigus and Pemphigoid". British Journal of Dermatology. 65 (12): 425-431. doi: ...
According to Pemphigus Pemphigoid Foundation (IPPF), "familial benign chronic pemphigus, or Hailey-Hailey disease, is a ... "Pemphigus". Pemphigus Pemphigold Foundation. 2014-03-19. Retrieved 29 October 2015. Foundation, British Skin. "Hailey-hailey ... The lack of oral lesions and intercellular antibodies distinguishes familial benign pemphigus from other forms of pemphigus.[ ... Hailey-Hailey disease (HHD), or familial benign chronic pemphigus: 559 or familial benign pemphigus,: 622 was originally ...
In an 1886 publication of Vierteljahrsschrift für Dermatologie und Syphilis, he described a type of pemphigus vulgaris, which ... First description of Mibelli's disease II). Ueber Pemphigus vegetans (frambosioides). in Vierteljahrsschrift für Dermatologie ... later became known as Pemphigus vegetans of Neumann. He was also the first to publish a detailed study (Über die senilen ...
"Canine Pemphigus Treatment with Prednisone". VetInfo. "Positional Cloning of the Gene(s) for Gastric Cancer in the Chow Chow". ... canine pemphigus, and gastric cancer. Chow Chows are a high risk breed for autoimmune disease and are at a predisposition for ...
Williams DM (December 1989). "Vesiculobullous mucocutaneous disease: pemphigus vulgaris". Journal of Oral Pathology and ... Pemphigus vulgaris Pemphigoid Dermatitis herpetiformis[1] Linear immunoglobulin-A disease (linear IgA disease) Genetic: ...
Nguyen VT, Ndoye A, Grando SA (Sep 2000). "Pemphigus vulgaris antibody identifies pemphaxin. A novel keratinocyte annexin-like ...
Kayani, Mahaz; Aslam, Arif M. (2017-06-08). "Bullous pemphigoid and pemphigus vulgaris". BMJ. 357: j2169. doi:10.1136/bmj.j2169 ... disease developed following bone marrow transplant in which its oral presentation is similar to Lichen Planus Pemphigus ...
Two months later, on May 11, 1916, his struggle with pemphigus may have led to his death at the age of 42. He rests in his ... While serving on the front in Russia in 1915, he began to suffer from pemphigus, a rare and painful autoimmune skin-disease. ... Pisanti, S.; Sharav, Y.; Kaufman, E.; Posner, L.N. (September 1974). "Pemphigus vulgaris: Incidence in Jews of different ethnic ... Vodo, Dan; Sarig, Ofer; Sprecher, Eli (14 August 2018). "The Genetics of Pemphigus Vulgaris". Frontiers in Medicine. 5: 226. ...
... ~clinical at eMedicine Serratos BD, Rashid RM (2009). "Nail disease in pemphigus vulgaris". Dermatol. Online J. 15 (7 ... Paronychia can occur with diabetes, drug-induced immunosuppression, or systemic diseases such as pemphigus. Paronychia aka " ...
Pemphigus vulgaris Pemphigus vegetans Pemphigus vegetans of Hallopeau Pemphigus vegetans of Neumann Pemphigus foliaceus, of ... pemphigus vulgaris (PV) and pemphigus foliaceus (PF). However, several other disorders such as IgA pemphigus, IgE pemphigus, ... the new variant endemic pemphigus Foliaeus and Tunisian endemic pemphigus foliaceus Paraneoplastic pemphigus IgA pemphigus, of ... The several types of pemphigus (pemphigus vulgaris, pemphigus foliaceus, intraepidermal neutrophilic IgA dermatosis, and ...
... consists of a group of rare and severe autoimmune blistering diseases mediated by pathogenic autoantibodies mainly ... The two main pemphigus variants are pemphigus vulgaris, which often originates with painful oral erosions, and pemphigus ... Pemphigus is diagnosed on the basis of either IgG or complement component 3 deposits (or both) at the keratinocyte cell ... Pemphigus consists of a group of rare and severe autoimmune blistering diseases mediated by pathogenic autoantibodies mainly ...
The availability of animal models of pemphigus has enabled researchers to understand the pathological mechanisms of this ... Pemphigus. Nature Reviews Disease Primers volume 3, Article number: 17027 (2017) Cite this article ... The availability of animal models of pemphigus has enabled researchers to understand the pathological mechanisms of this ... Pemphigus. Nat Rev Dis Primers 3, 17027 (2017). https://doi.org/10.1038/nrdp.2017.27 ...
Pemphigus vulgaris (PV) is an autoimmune disorder of the skin. It involves blistering and sores (erosions) of the skin and ... Pemphigus vulgaris (PV) is an autoimmune disorder of the skin. It involves blistering and sores (erosions) of the skin and ... Severe cases of pemphigus may need wound management, similar to the treatment for severe burns. People with PV may need to stay ... Pemphigus vulgaris (PV) is an autoimmune disorder of the skin. It involves blistering and sores (erosions) of the skin and ...
The conventional treatment mainstays for pemphigus are problematic during the COVID-19 pandemic, and a shift in disease ... The absence of this immunologic protection for such a long time poses potentially serious problems for pemphigus patients who ... Another option is to consider enrolling a patient with moderate or severe pemphigus vulgaris or foliaceus in the ongoing ... First off, rituximab (Rituxan), the only Food and Drug Administration-approved medication for moderate to severe pemphigus ...
Unlike typical immunoglobulin G (IgG)-mediated pemphigus, IgA pemphigus is characterized by tissue-bound and circulating IgA ... pemphigus is a group of newly characterized immune-mediated intraepidermal blistering skin diseases. ... Non-classical forms of pemphigus: pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus. An ... Pemphigus Foliaceus, Pemphigus Herpetiformis, Pemphigus Vulgaris, Drug-Induced Pemphigus, and Paraneoplastic Pemphigus. ...
Pemphigus is a rare group of autoimmune diseases. It causes blisters on the skin and mucous membranes throughout the body. It ... Pemphigus vulgaris is the most common type of pemphigus.. What causes pemphigus vulgaris?. Pemphigus vulgaris is not fully ... What is pemphigus vulgaris?. Pemphigus is a rare group of autoimmune diseases. It causes blisters on the skin and mucous ... How is pemphigus vulgaris treated?. These are common treatments for pemphigus vulgaris:. *Better oral health care. Blistering ...
Learn about how dermatologists identify the signs and symptoms of pemphigus. ... Pemphigus foliaceus. This type of pemphigus affects only the skin and often develops on the face, scalp, and upper body. ... Pemphigus vulgaris. The blisters have burst, leaving painful sores. About 70% of people who get pemphigus have this type. ... Drug-induced pemphigus. Some people get pemphigus after taking certain medicines. This man took penicillamine, which is the ...
Treatments and Tools for pemphigus. Find pemphigus information, treatments for pemphigus and pemphigus symptoms. ... pemphigus - MedHelps pemphigus Center for Information, Symptoms, Resources, ... Is pemphigus foliaceus contagious and how long does it take to disappear? is it a form of ... ... Hi, Can you help me with the most common treatments for Pemphigus foliaceus? And how long w... ...
PEMPHIGUS VULGARIS. Shift in clinical features, histologic findings and antigen profiles from pemphigus vulgaris to pemphigus ... Treatment of pemphigus vulgaris and pemphigus foliaceus: experience with 71 patients over a 20 year period. ... in pemphigus vulgaris or paraneoplastic pemphigus. However, erythrodermic forms of the endemic variant of pemphigus foliaceus ... Clinical and Serological Transition From Pemphigus Vulgaris to Pemphigus Foliaceus Demonstrated by Desmoglein ELISA System ...
Pemphigus in domestic animals. Pemphigus foliaceus skin eruption on the ventral abdomen of a dog. Pemphigus foliaceus has been ... There are three types of pemphigus which vary in severity: pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic ... Pemphigus vulgaris is a very rare disorder described in pet dogs and cats. Paraneoplastic pemphigus has been identified in pet ... Pemphigus Vulgaris. *. Ahmed AR, Spigelman Z, Cavacini LA, Posner MR (2006). "Treatment of pemphigus vulgaris with rituximab ...
Paraneoplastic pemphigus pathology. Authoritative facts about the skin from DermNet New Zealand. ... Paraneoplastic pemphigus is a rare form of pemphigus associated with internal malignancies. Lymphoma is the most commonly ... Histology of paraneoplastic pemphigus. In paraneoplastic pemphigus, there is usually an overt interface or lichenoid infiltrate ... Paraneoplastic pemphigus pathology. Author: Assoc Prof Patrick Emanuel, Dermatopathologist, Auckland, New Zealand, 2014. ...
What Is Paraneoplastic Pemphigus?. Paraneoplastic Pemphigus is a life threatening autoimmune mucocutaneous, blistering disease ... What Are The Symptoms Of Paraneoplastic Pemphigus?. Patients with paraneoplastic pemphigus display the presence of lesions ... "Pemphigus-like": causing blister crust over the raw exuding skin lesion.. *"Pemphigoid-like": severe blister leading to redness ... What Causes Paraneoplastic Pemphigus?. According to various l conducted research to ascertain the cause of paraneoplastic ...
Merck & Co., Inc., Rahway, NJ, USA (known as MSD outside of the US and Canada) is dedicated to using the power of leading-edge science to save and improve lives around the world. The Merck Veterinary Manual was first published in 1955 as a service to the community. The legacy of this great resource continues as the Merck Veterinary Manual in the US and Canada and the MSD Vet Manual outside of North America.. ...
Tham HL, Linder KE, Olivry T. Deep pemphigus (pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus) in dogs, ... The pemphigus foliaceous variant of the pemphigus complex is caused by autoantibodies targeting a specific component of ... Pemphigus foliaceous can make this tricky in the cat, as this type of disease commonly waxes and wanes, can be rapid or very ... Feline pemphigus foliaceous is the most common autoimmune skin disease recognized in cats and most commonly presents as a ...
Anhalt et al first described paraneoplastic pemphigus in 1990. The authors reported 5 patients with underlying neoplasms who ... Paraneoplastic pemphigus is the only form of pemphigus that affects epithelia other than squamous. Involvement of respiratory ... Paraneoplastic pemphigus associated with Castleman tumor: a commonly reported subtype of paraneoplastic pemphigus in China. ... Ishii N, Hashimoto T. A case of paraneoplastic pemphigus who shifted to pemphigus vulgaris. J Eur Acad Dermatol Venereol. 2008 ...
... also called Brazilian Pemphigus, South American Pemphigus, endemic Pemphigus, wildfire, "fuego salvaje", "fogo selvagem") ... Pemphigus foliaceous (PF) is undeniably the most common pemphigus variety worldwide. In human medicine, PF has two distinct ... The Pemphigus complex (PC) belongs to a group of autoimmune illnesses, with increasing worldwide prevalence but in the last ... Pemphigus Complex (Wildfire): Epidemiology and Clinics World Small Animal Veterinary Association World Congress Proceedings, ...
Efgartigimod alfa is under clinical development by Argenx and currently in Phase III for Pemphigus Vulgaris. ... Premium Insights Likelihood of Approval and Phase Transition Success Rate Model - Efgartigimod alfa in Pemphigus Vulgaris Buy ... Premium Insights Likelihood of Approval and Phase Transition Success Rate Model - Efgartigimod alfa in Pemphigus Vulgaris. Buy ... Efgartigimod alfa is under clinical development by Argenx and currently in Phase III for Pemphigus Vulgaris. According to ...
Periplakin, an autoantigen in paraneoplastic pemphigus and pemphigus foliaceus, an intermediate filament-binding protein, and a ... Kazerounian, Shideh, "Periplakin, an autoantigen in paraneoplastic pemphigus and pemphigus foliaceus, an intermediate filament- ...
The differentials for PV cutaneous lesions are mistaken for pemphigus foliaceus, pemphigus vegetans, IgA pemphigus, ... paraneoplastic pemphigus, bullous pemphigoid, linear IgA disease, erythema multiforme, Grover disease, and Hailey-Hailey ... Pemphigus vulgaris is a rare autoimmune disease and many health practitioners are still unaware of it. Due to its low incidence ... Pemphigus foliaceus; Pemphigus erythematosus; Paraneoplastic pemphigus; Herpetiform pemphigus; Drug-induced pemphigus; IgA ...
Pulse therapy in pemphigus and other diseases. 2nd Ed, New Delhi: Pulse Therapy and Pemphigus Foundation, 2000.. 14. Rosenberg ... Twenty-five patients had pemphigus vulgaris, and 1 had pemphigus vegetans, the duration of illness was 2 months to 12 years ( ... Twenty-six patients (12 men, 14 women; mean age, 48.4 years), comprising 25 cases with pemphigus vulgaris and 1 with pemphigus ... clears lesions of pemphigus with , 10 percent body surface involvement, and this may be an alternative regimen for pemphigus. ...
... blistering disorders such as pemphigus and phemphigoid. We will provide you with quality care and the best available treatments ... Pemphigus Types. The two most common types of pemphigus are:. *Pemphigus vulgaris - This begins with painful blisters in your ... Pemphigus vs. Pemphigoid Pemphigoid is another autoimmune skin disorder that bears a striking resemblance to pemphigus because ... IgA pemphigus - This is the least harmful type of pemphigus that can cause small, pus-fillled bumps. The blisters bear a ...
This study group consisted of nine pemphigus vulgaris (PV), as well as three pemphigus foliaceus (PF) patients who were ... Strikingly, in pemphigus patients, a significant correlation was not only found between serum IgG auto-ab titers and plasma ... Similarly, active pemphigus patients displayed an upregulation of CCR2 on blood CD14+ monocytes, as well as elevated numbers of ... Characterization of cellular and humoral immune responses in pemphigus patients and an HLA-transgenic mouse model The rare, but ...
Neumann pemphigus vegetans was the most common form in 11/17 patients; lesions were multifocal in 16/17 patients and mainly ... Study of pemphigus vegetans finds no correlation between clinical findings and prognostic outcome. ... Hospital prevalence was 0.084% with a frequency of 9.1% among patients with pemphigus. Mean age at presentation was 47.6 years ... A Tunisian retrospective study (1981-2009) of 17 cases of pemphigus vegetans was conducted to analyze epidemiological, clinical ...
Whenever pemphigus vulgaris and erythema multiforme are part of a differential diagnosis, paraneoplastic pemphigus should be ... Pemphigus is a very common bullous disorder in India. At any point there are 4-5 patients admitted at our centre for management ... Pemphigus vulgaris is a potentially fatal autoimune blistering disease. He was admitted for inpatient treatment with IV fluids ... Pemphigus vulgaris is relatively a common social & medical problem in Iraq, often affecting young adult people. Most patients ...
Pemphigus vulgaris localised exclusively to the penis. Ozlem Su1 , Didem Dizman1 , Dilek Biyik Ozkaya1 , Pelin Yildiz2 , Cuyan ... 2],[3] Sami and Ahmet reported involvement of the penile skin in 12 male patients with pemphigus. However, in none of these ... Pemphigus vulgaris is a chronic, autoimmune bullous disease affecting the mucosae and skin. Though disease usually starts in ... Su O, Dizman D, Ozkaya DB, Yildiz P, Demirkesen C, Onsun N. Pemphigus vulgaris localised exclusively to the penis. Indian J ...
... in adults with moderate to severe pemphigus vulgaris (PV). The study met the primary endpoint at week 52 and demonstrated that ... International Pemphigus & Pemphigoid Foundation. 915 Highland Pointe Dr, Ste 250. Roseville, CA 95678. United States of America ... "The approval of Rituxan for the treatment of pemphigus vulgaris was the first major advancement in the treatment of this rare, ... The International Pemphigus & Pemphigoid Foundations most important objectives are to provide patients and doctors worldwide ...
IGA score of pemphigus severity, possible score from 0-4. *Pemphigus Disease Activity Index (PDAI) Score [ Time Frame: Baseline ... Validation of 5-Point Investigator Global Assessments for Pemphigus. The safety and scientific validity of this study is the ... Photograph sets of pemphigus lesions will be gathered from the lead site and participant sites. They will be de-identified and ... In the first part of the study, 8 dermatologists will score 20 sets of photographs of pemphigus lesions (a mixture of skin and ...
Herbal Medicine for Pemphigus. $99.00. Burdock, Golden Seal, Fenugreek, Liquorice, Vervain, Rosehip, Elecampane, Hypericum and ...
... and Epitope Characteristics Do Not Differ in IgG Subclass-Switched Anti-Desmoglein 3 IgG1 and IgG4 Autoantibodies in Pemphigus ... and Epitope Characteristics Do Not Differ in IgG Subclass-Switched Anti-Desmoglein 3 IgG1 and IgG4 Autoantibodies in Pemphigus ... and Epitope Characteristics Do Not Differ in IgG Subclass-Switched Anti-Desmoglein 3 IgG1 and IgG4 Autoantibodies in Pemphigus ...
  • The several types of pemphigus (pemphigus vulgaris, pemphigus foliaceus, intraepidermal neutrophilic IgA dermatosis, and paraneoplastic pemphigus) vary in severity. (wikipedia.org)
  • citation needed] Pemphigus foliaceus (PF) is the least severe of the three varieties. (wikipedia.org)
  • Endemic pemphigus foliaceus includes fogo selvagem, the new variant of endemic pemphigus foliaceus in El Bagre, Colombia, and the Tunisian endemic pemphigus in North Africa. (wikipedia.org)
  • Pemphigus is further divided in two major subtypes: pemphigus vulgaris (PV) and pemphigus foliaceus (PF). (wikipedia.org)
  • However, several other disorders such as IgA pemphigus, IgE pemphigus, pemphigus herpetiformis, drug-induced pemphigus, Senear Usher syndrome, and endemic pemphigus foliaceus exist, and are recognized by a dermatologist from the appearance and distribution of the skin lesions. (wikipedia.org)
  • The two main pemphigus variants are pemphigus vulgaris, which often originates with painful oral erosions, and pemphigus foliaceus, which is characterised by exclusive skin lesions. (nih.gov)
  • Another option is to consider enrolling a patient with moderate or severe pemphigus vulgaris or foliaceus in the ongoing pivotal phase 3, international, double-blind, placebo-controlled PEGASUS trial of rilzabrutinib, a promising oral reversible Bruton tyrosine kinase inhibitor. (medscape.com)
  • Those with desmoglein-1 or desmoglein-3 target antigens are diagnosed with having IgA pemphigus foliaceus (IgA-PF) or IgA pemphigus vulgaris (IgA-PV), respectively. (medscape.com)
  • Although the term IgA pemphigus has not been established in the veterinary literature, IgA deposition around epidermal cells has been detected by direct immunofluorescence in animals affected with pemphigus foliaceus. (medscape.com)
  • Medscape articles on other variants of pemphigus include Pemphigus Erythematosus , Pemphigus Foliaceus , Pemphigus Herpetiformis , Pemphigus Vulgaris , Drug-Induced Pemphigus , and Paraneoplastic Pemphigus . (medscape.com)
  • Is pemphigus foliaceus contagious and how long does it take to disappear? (medhelp.org)
  • Hi, Can you help me with the most common treatments for Pemphigus foliaceus? (medhelp.org)
  • There is an interesting infectious variant, termed endemic pemphigus foliaceus or fogo selavagem. (thedoctorsdoctor.com)
  • Detection of mercury and other undetermined materials in skin biopsies of endemic pemphigus foliaceus. (thedoctorsdoctor.com)
  • A novel variant of endemic pemphigus foliaceus (EPF) was described among individuals in an area surrounding El Bagre, Colombia, South America. (thedoctorsdoctor.com)
  • There are three types of pemphigus which vary in severity: pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus. (wikidoc.org)
  • Pemphigus foliaceus is characterized by crusty sores that often begin on the scalp , and may move to the chest, back, and face. (wikidoc.org)
  • They can also exhibit antibodies to antigens associated with pemphigus vulgaris (desmoglein 3, 130-kd) and pemphigus foliaceus (desmoglein 1, 160-kd), as well as several others, including epiplakin. (medscape.com)
  • When the cleavage process is high, with the involvement of the granular or the sub-corneal layer, it is classified as superficial pemphigus (foliaceus or erythematosus). (vin.com)
  • Kazerounian, Shideh, "Periplakin, an autoantigen in paraneoplastic pemphigus and pemphigus foliaceus, an intermediate filament-binding protein, and a component of the insoluble structures of epidermal keratinocytes" (2002). (jefferson.edu)
  • Pemphigus foliaceus - These itchy blisters form on your back, chest and shoulders, but not in your mouth. (utah.edu)
  • The blisters bear a striking resemblance to pemphigus foliaceus, but they are actually caused by the IgA antibody. (utah.edu)
  • This study group consisted of nine pemphigus vulgaris (PV), as well as three pemphigus foliaceus (PF) patients who were categorized according to either active or remittent state of disease. (uni-marburg.de)
  • Although rare, cats can suffer from autoimmune skin diseases which includes pemphigus foliaceus, pemphigus vulgaris, pemphigus erythematosus and paraneoplastic pemphigus. (animalwised.com)
  • it is considered an intermediate form between pemphigus erythematosus and pemphigus foliaceus. (animalwised.com)
  • In cats, pemphigus foliaceus is the most common autoimmune disease . (animalwised.com)
  • Yellowish crusts are more frequently observed in cats when it comes to pemphigus foliaceus. (animalwised.com)
  • Due to the itching produced by pemphigus foliaceus in cats, a differential diagnosis must be made between other diseases that cause itching in this species, such as allergies and parasitic diseases. (animalwised.com)
  • Pemphigus foliaceus doesn't cause mouth blisters. (ahdubai.com)
  • Pemphigus foliaceus (PF) is a type of autoimmune disease of the skin. (vin.com)
  • What Species or Breeds get Pemphigus Foliaceus? (vin.com)
  • How is Pemphigus Foliaceus Treated? (vin.com)
  • How Long Does My Pet Need to be Treated for Pemphigus Foliaceus? (vin.com)
  • A mixed immunoblistering disorder exhibiting features of bullous pemphigoid and pemphigus foliaceus (PF), associated with the food supplement Spirulina, was described in an 82-year-old woman. (medscape.com)
  • Materiali K.uchenigu o pemphigus foliaceus [doctoral thesis]. (medscape.com)
  • Aoki V, Rivitti EA, Diaz LA. Update on fogo selvagem, an endemic form of pemphigus foliaceus. (medscape.com)
  • Abreu-Velez AM, Beutner EH, Montoya F, Bollag WB, Hashimoto T. Analyses of autoantigens in a new form of endemic pemphigus foliaceus in Colombia. (medscape.com)
  • Paraneoplastic pemphigus with cutaneous and serological features of pemphigus foliaceus. (medscape.com)
  • Tunisian pemphigus foliaceus with antidesmoglein 3 antibody]. (medscape.com)
  • Protection against pemphigus foliaceus by desmoglein 3 in neonates. (medscape.com)
  • Inadomi T. A case of pemphigus foliaceus aggravated in an irradiated area by radiotherapy against breast cancer. (medscape.com)
  • Liebman TN, Lieberman MR, Burris K. Pemphigus foliaceus exacerbated by radiation, in association with myasthenia gravis. (medscape.com)
  • Brochado MJ, Nascimento DF, Campos W, Deghaide NH, Donadi EA, Roselino AM. Differential HLA class I and class II associations in pemphigus foliaceus and pemphigus vulgaris patients from a prevalent Southeastern Brazilian region. (medscape.com)
  • Pigozzi B, Peserico A, Schiesari L, Alaibac M. Pemphigus foliaceus evolving into pemphigus vulgaris: a probable example of 'intermolecular epitope spreading' confirmed by enzyme-linked immunosorbent assay study. (medscape.com)
  • Dalla-Costa R, Pincerati MR, Beltrame MH, Malheiros D, Petzl-Erler ML. Polymorphisms In the 2q33 and 3q21 Chromosome Regions Including T Cell Coreceptor and Ligand Genes May Influence Susceptibility to Pemphigus Foliaceus. (medscape.com)
  • Autoimmune reactivity against precursor form of desmoglein 1 in healthy Tunisians in the area of endemic pemphigus foliaceus. (medscape.com)
  • We report a patient with known pemphigus foliaceus who had significant epidermal detachment of normal skin during the removal of patch-testing tape. (medscape.com)
  • Cite this: Pemphigus Foliaceus with Epidermal Detachment: Adverse Events from Patch Testing - Medscape - Mar 01, 2006. (medscape.com)
  • Ivy's biopsy came back yesterday with a positive result for a rare auto immune disease called Pemphigus Foliaceus. (troixmagazine.com)
  • Pemphigus foliaceus is the most common form of presentation in dogs. (dogsis.com)
  • If the disease resolves when the drug is withdrawn, it is called a "pemphigus foliaceus-type drug reaction," and if the disease persists after drug withdrawal, it is called a "drug-induced foliaceous pemphigus. (dogsis.com)
  • Pemphigus foliaceus is a disease whose symptoms appear progressively, giving rise to formation of vesicles or pustules, redness of the skin, peeling, hair loss and evident appearance of crusts . (dogsis.com)
  • Furthermore, some studies suggest a high incidence of pemphigus foliaceus in allergic dogs. (dogsis.com)
  • Demodicosis, superficial pyoderma, dermatophytosis, discoid lupus erythematosus, rash due to drugs, are some of the processes that have clinical signs similar to those that occur in pemphigus foliaceus and erythematosus. (dogsis.com)
  • desmoglein 1 antibodies in endemic areas to pemphigus foliaceus: A case series. (bvsalud.org)
  • Ultrastructural skin alterations of healthy subjects with anti -desmoglein 1 antibodies in endemic areas to pemphigus foliaceus: A case series. (bvsalud.org)
  • Endemic pemphigus foliaceus and endemic pemphigus vulgaris are autoimmune dermatologic disorders endemic to the Peruvian Amazon . (bvsalud.org)
  • To determine the ultrastructural skin alterations of three healthy subjects with anti DSG-1 antibodies in areas endemic to pemphigus foliaceus and pemphigus vulgaris in the Peruvian Amazon . (bvsalud.org)
  • The least common and most severe type of pemphigus is paraneoplastic pemphigus (PNP). (wikipedia.org)
  • Pemphigus vulgaris is the most common type of pemphigus. (hopkinsmedicine.org)
  • This type of pemphigus affects only the skin and often develops on the face, scalp, and upper body. (aad.org)
  • Painful mouth sores are common in people who have pemphigus vulgaris, the most common type of pemphigus. (aad.org)
  • IgA pemphigus - This is the least harmful type of pemphigus that can cause small, pus-fillled bumps. (utah.edu)
  • We can determine the type of pemphigus by running on the skin sample. (utah.edu)
  • This type of pemphigus is accompanied by systemic signs such as fever, depression and loss of appetite. (dogsis.com)
  • Severe cases of pemphigus may need wound management, similar to the treatment for severe burns . (medlineplus.gov)
  • In 2007, a clinical trial including a one-week treatment with Rituximab , a monoclonal chimaeric Anti-CD20-Antibody, approved by the FDA for the treatment of B-Cell-Non-Hodgkin-Lymphoma and severe cases of rheumatoid arthritis , showed a remission in 18 of 21 otherwise untreatable, severe cases of Pemphigus vulgaris. (wikidoc.org)
  • Pulse intravenous cyclophosphamide without a concomitant corticosteroid pulse has also been used in recalcitrant cases of pemphigus with encouraging results [ 7 , 8 ]. (cdlib.org)
  • A Tunisian retrospective study (1981-2009) of 17 cases of pemphigus vegetans was conducted to analyze epidemiological, clinical, immunopathological and therapeutic data. (accessdermatology.com)
  • People with severe cases of pemphigus vulgaris may need to be hospitalized to get wound care and intravenous (IV) fluids or electrolytes if mouth sores make it difficult to eat and drink. (franciscanhealth.org)
  • However, homeopathy is strongly indicated in most cases of pemphigus as a support measure, which helps not only to manage the disease better, but also helps to reduce the dependency of cortisone. (drankireddy.com)
  • In this AnimalWised article we're going to talk about autoimmune skin disease in cats, including the different types of pemphigus in cats . (animalwised.com)
  • There are different types of pemphigus in cats. (animalwised.com)
  • In 1964, researchers found that the blood of patients with pemphigus contained antibodies to the layers of skin that separate to form the blisters. (wikipedia.org)
  • Pemphigus vulgaris (PV - ICD-10 L10.0) is the most common form of the disorder and occurs when antibodies attack desmoglein 3. (wikipedia.org)
  • Pemphigus is diagnosed on the basis of either IgG or complement component 3 deposits (or both) at the keratinocyte cell membrane, detected by direct immunofluorescence microscopy of a perilesional biopsy, with serum anti-Dsg1 or anti-Dsg3 antibodies (or both) detected by ELISA. (nih.gov)
  • The presence of these antibodies indicates pemphigus. (wikidoc.org)
  • Passive transfer of paraneoplastic pemphigus sera causes blistering in neonatal mice, proving that the antibodies present are pathogenic. (medscape.com)
  • We may also draw your blood to measure the levels of pemphigus antibodies in your system. (utah.edu)
  • But in pemphigus, the body produces antibodies that damage cells of your skin and mucous membranes. (ahdubai.com)
  • One purpose of these tests is to detect and identify antibodies in your blood that are known to be present with pemphigus. (ahdubai.com)
  • Pemphigus antibodies bound to both mouse and human cultured epidermal cells. (duke.edu)
  • These intercellular or pemphigus vulgaris antibodies bind to keratinocyte desmosomes and to desmosome-free areas of the keratinocyte cell membrane. (diseasesdic.com)
  • However, in an autoimmune disease, such as pemphigus vulgaris, the immune system makes antibodies (autoantibodies) that work against tissues in the body. (diseasesdic.com)
  • Although it is known that antibodies to desmoglein cause pemphigus Vulgaris, it is still not clear why some people develop these autoantibodies. (diseasesdic.com)
  • Pemphigus antibodies identify a cell surface glycoprotein synthesized by human and mouse keratinocytes. (vestnikdv.ru)
  • A diagnosis of neoplastic pemphigus may prompt a search for an existing tumor. (wikidoc.org)
  • Histopathology is critical for an accurate diagnosis of pemphigus foliaceous in cats. (mspca.org)
  • A Clinician's Guide to Pemphigus Vulgaris: Differential Diagnosis of Cutaneous Lesions Mohammad Beigi, Pooya Khan 2017-11-27 00:00:00 [Pemphigus vulgaris is a rare autoimmune disease and many health practitioners are still unaware of it. (deepdyve.com)
  • A Clinician's Guide to Pemphigus Vulgaris Differential Diagnosis of Cutaneous Lesions %22&body=%0AI%20found%20an%20article%20you%20might%20be%20interested%20in. (deepdyve.com)
  • Nonhematological autoimmunity (glomerulosclerosis, paraneoplastic pemphigus and paraneoplastic neurological syndrome) in a patient with chronic lymphocytic leukemia: Diagnosis, prognosis and management. (medscape.com)
  • Pemphigus Vulgaris can cause very extensive, life-threatening erosions, especially if the diagnosis is delayed. (diseasesdic.com)
  • BACKGROUND: Endemic forms of pemphigus are a unique group of autoimmune diseases that represent opportunities to study interactions of the environment and genetics with the immune system. (thedoctorsdoctor.com)
  • Recently, there has been great promise of surviving some forms of pemphigus (especially PNP) by using a pooled blood product known as gamma globulin or IVIG. (wikidoc.org)
  • Unlike other forms of pemphigus, paraneoplastic pemphigus affects organ systems other than the integument. (medscape.com)
  • The more common of the two forms of pemphigus, which is an autoimmune disease that causes blisters on your skin and mucous membranes to rupture, creating open sores that may become infected. (autoimmuneinstitute.org)
  • Some people find that stress and certain foods, such as garlic, make living with pemphigus vulgaris more difficult. (hopkinsmedicine.org)
  • This PrimeView illustrates how autoantibodies against proteins that are involved in cell adhesion can lead to the formation of blisters and erosions, the hallmark lesions of pemphigus. (nature.com)
  • If left untreated, pemphigus vulgaris can cause life-threatening erosions in the skin, mucosal membranes, and other tissues. (autoimmuneinstitute.org)
  • This causes blisters and erosions that are typical of pemphigus Vulgaris. (diseasesdic.com)
  • The lesions that accompany the erythematous pemphigus consist of pustules that develop into ulcers, erosions and crusts . (dogsis.com)
  • True (acantholytic) pemphigus is an autoimmune disease characterized by blisters and erosions in the skin and/or mucous tunics. (vestnikdv.ru)
  • This man took penicillamine, which is the most common cause of drug-induced pemphigus. (aad.org)
  • Patients with vegetating lesions and pemphigus vegetans-like histology are classified as IgA pemphigus vegetans (IgA-PVeg). (medscape.com)
  • Pemphigus vegetans - Thick sores in your groin and armpits are a tell-tale sign of this kind of pemphigus. (utah.edu)
  • Chorzelski T, Jablonska S, Blaszczyk M. Immunopathological investigations in the Senear-Usher syndrome (coexistence of pemphigus and lupus erythematosus). (medscape.com)
  • Systemic lupus erythematosus, drug eruptions, skin neoplasia, mucocutaneous candidiasis, are some of the pathologies with symptoms similar to those described for pemphigus vulgaris and paraneoplastic. (dogsis.com)
  • If your doctor suspects you have pemphigus vulgaris or bullous pemphigoid, an endoscopy may be performed to check for sores in your throat. (utah.edu)
  • This study aims to obtain statistical data regarding the inter-rater and intra-rater reliability of the IGAs, Pemphigus Disease Activity Index (PDAI) and Autoimmune Bullous Skin Disease Intensity Score (ABSIS) as well as the convergent validity of the IGAs with the PDAI and objective component of the ABSIS. (clinicaltrials.gov)
  • Pemphigus is distinct from bullous pemphigoid, which is a blistering skin condition that affects older adults and may cause death. (ahdubai.com)
  • Coexistence of paraneoplastic pemphigus and bullous pemphigoid. (medscape.com)
  • ABSTRACT Molecular mechanisms underlying the pathophysiology of pemphigus vulgaris are still not clear. (who.int)
  • In human medicine, PF has two distinct forms: the classic or European described by Cazenave in 1844 (Cazenave Pemphigus) and the endemic (also called Brazilian Pemphigus, South American Pemphigus, endemic Pemphigus, wildfire, "fuego salvaje", "fogo selvagem") reported by Vieira in 1940. (vin.com)
  • Endemic Pemphigus, also called Fogo Selvagem, is a very rare blistering disease of the skin that I rarely ever encounter in either my The Woodlands dermatology and Conroe dermatology offices. (perridermatology.com)
  • First off, rituximab ( Rituxan ), the only Food and Drug Administration-approved medication for moderate to severe pemphigus vulgaris and a biologic considered first-line therapy prepandemic, is ill-advised during the COVID-19 era. (medscape.com)
  • Genentech , a member of the Roche Group, announced data this week from the Phase III PEMPHIX study evaluating the efficacy and safety of Rituxan® (rituximab) compared to mycophenolate mofetil (MMF) in adults with moderate to severe pemphigus vulgaris (PV). (pemphigus.org)
  • In pemphigus, autoantibodies form against desmoglein, which forms the "glue" that attaches adjacent epidermal cells via attachment points called desmosomes. (wikipedia.org)
  • Pemphigus consists of a group of rare and severe autoimmune blistering diseases mediated by pathogenic autoantibodies mainly directed against two desmosomal adhesion proteins, desmoglein (Dsg)1 and Dsg3 (also known as DG1 and DG3), which are present in the skin and surface-close mucosae. (nih.gov)
  • The rare, but potentially fatal autoimmune disorder pemphigus is considered as a prototypical antibody-mediated organ-specific disease, in which immunoglobulin (Ig) G autoantibodies (auto-ab) mainly target the desmosomal cadherins Desmoglein 3 (Dsg3) and Dsg1 within the epidermis. (uni-marburg.de)
  • Pathogenicity and Epitope Characteristics Do Not Differ in IgG Subclass-Switched Anti-Desmoglein 3 IgG1 and IgG4 Autoantibodies in Pemphigus Vulgaris. (umassmed.edu)
  • Lo AS, Mao X, Mukherjee EM, Ellebrecht CT, Yu X, Posner MR, Payne AS, Cavacini LA. Pathogenicity and Epitope Characteristics Do Not Differ in IgG Subclass-Switched Anti-Desmoglein 3 IgG1 and IgG4 Autoantibodies in Pemphigus Vulgaris. (umassmed.edu)
  • Desmoglein 1 and desmoglein 3 are the target autoantigens in herpetiform pemphigus. (medscape.com)
  • Herpetiform pemphigus showing reactivity with pemphigus vulgaris antigen (desmoglein 3). (medscape.com)
  • When the autoantibodies formed in pemphigus vulgaris attack the desmoglein proteins, the cells in the skin and mucous membranes no longer hold together and separate. (diseasesdic.com)
  • Pemphigus (/ˈpɛmfɪɡəs/ or /pɛmˈfaɪɡəs/) is a rare group of blistering autoimmune diseases that affect the skin and mucous membranes. (wikipedia.org)
  • Pemphigus is a rare group of autoimmune diseases that causes blisters on the skin and mucous membranes throughout the body. (hopkinsmedicine.org)
  • Pemphigus is an autoimmune disorder that causes blistering and raw sores on skin and mucous membranes . (wikidoc.org)
  • Pemphigus can manifest itself as blisters and sores on your skin or mucous membranes of the body like your mouth, eyes, nose, throat, and genitals. (utah.edu)
  • Pemphigoid is another autoimmune skin disorder that bears a striking resemblance to pemphigus because blisters and rashes can appear on the skin and mucous membranes of the body. (utah.edu)
  • Pemphigus is a disease that causes blisters and sores on the skin or mucous membranes, such as in the mouth or on the genitals. (ahdubai.com)
  • Pemphigus causes blisters on your skin and mucous membranes. (ahdubai.com)
  • The general designation of pemphigus covers a number of autoimmune pathologies characterized by the development of intraepithelial blisters on the skin and mucous membranes. (bvsalud.org)
  • Pemphigus Vulgaris is an autoimmune disorder that involves blistering and erosion of the skin and mucous membranes. (diseasesdic.com)
  • Pemphigus vulgaris is an autoimmune, intraepithelial, blistering disease affecting the skin and mucous membranes and is mediated by circulating autoantibodies directed against keratinocyte cell surfaces. (diseasesdic.com)
  • Pemphigus vulgaris is a rare, severe autoimmune disorder in which blisters of varying sizes break out on the skin and on the lining of the mouth and other mucous membranes. (msdmanuals.com)
  • Pemphigus Vulgaris is an autoimmune condition of the skin characterized by blister formation on the skin and mucous membranes. (homeodoctor.co.in)
  • The conventional treatment mainstays for pemphigus are problematic during the COVID-19 pandemic, and a shift in disease management strategy is in order, Dedee F. Murrell, MD, said at the virtual annual meeting of the American Academy of Dermatology. (medscape.com)
  • It included 26 patients of pemphigus who presented to the dermatology outpatient department at a tertiary care hospital in New Delhi, India. (cdlib.org)
  • A biopsy is essential to diagnose pemphigus along with awareness of the clinical characteristics of these lesions. (bvsalud.org)
  • Doctors can diagnose pemphigus vulgaris by examining skin samples under a microscope. (msdmanuals.com)
  • To diagnose pemphigus, a complex of studies is needed taking into consideration the body of clinical symptoms and laboratory indices. (vestnikdv.ru)
  • Although it has long been recognized that auto-ab crucially contribute to the pathology of pemphigus, the exact immunological events leading to the loss of self-tolerance have not yet been fully identified. (uni-marburg.de)
  • Familial Benign Pemphigus Pathology. (vohrawoundcare.com)
  • DermNet NZ, 2015, dermnetnz.org/topics/familial-benign-pemphigus-pathology. (vohrawoundcare.com)
  • Mycophenolate is effective in the treatment of pemphigus vulgaris. (vgrd.org)
  • Oral cyclophosphamide for the treatment of pemphigus vulgaris and foliceous. (vgrd.org)
  • The approval of Rituxan for the treatment of pemphigus vulgaris was the first major advancement in the treatment of this rare, serious disease in more than 60 years, " said Levi Garraway, M.D., Ph.D., chief medical officer and head of Global Product Development. (pemphigus.org)
  • The treatment of Pemphigus vulgaris involves the administration of oral and systemic corticosteroids in the early stages to control the disease, which, if handled improperly, can be fatal. (bvsalud.org)
  • The autoantibodies in pemphigus vulgaris attack proteins called desmogleins. (diseasesdic.com)
  • Hematologic malignancies are most commonly associated with paraneoplastic pemphigus, although it can also be associated with carcinomas, sarcomas, and benign neoplasms. (medscape.com)
  • Autoantibody production from a thymoma and a follicular dendritic cell sarcoma associated with paraneoplastic pemphigus. (medscape.com)
  • Paraneoplastic pemphigus (paraneoplastic autoimmune multiorgan syndrome): clinical presentations and pathogenesis. (medscape.com)
  • Long term complete remission of severe pemphigus vulgaris with monoclonal anti CD20 antibody therapy and immunophenotype correlation. (vgrd.org)
  • Incubation of cultured newborn mouse epidermal cells with pemphigus antibody followed by gentle agitation induced loss of adhesion between the epidermal cells and the plastic culture dish. (duke.edu)
  • These observations suggest that pemphigus antibody induces viable epidermal cells to activate cellular proteinases which then degrade the glycocalyx and cause cellular dyshesion and acantholysis. (duke.edu)
  • Paraneoplastic Pemphigus is a life threatening autoimmune mucocutaneous, blistering disease triggered by malignant neoplasm. (spts.org)
  • 1] The disease can be occasionally associated with a paraneoplastic pemphigus (PNP), an autoimmune mucocutaneous disorder commonly seen in neoplasms of lymphocytic origin. (ejmo.org)
  • Originally, the cause of this disease was unknown, and "pemphigus" was used to refer to any blistering disease of the skin and mucosa. (wikipedia.org)
  • In this variety of pemphigus, the disease process often involves the lungs, causing bronchiolitis obliterans (constrictive bronchiolitis). (wikipedia.org)
  • Hailey-Hailey disease, also called familial benign pemphigus, is an inherited skin disease, not an autoimmune disease, so it is not considered part of the pemphigus group of diseases. (wikipedia.org)
  • The availability of animal models of pemphigus has enabled researchers to understand the pathological mechanisms of this autoimmune disease. (nature.com)
  • OBJECTIVES: The aims of this study were to confirm the endemic nature of a new type of autoimmune disease occurring in a mining town in northeastern Colombia in the El Bagre area, to characterize it, and to compare it with other forms of endemic pemphigus. (thedoctorsdoctor.com)
  • In this variety of pemphigus, the disease process often involves Bronchiolitis obliterans , a fatal destruction of alveoli in lung tissue. (wikidoc.org)
  • Feline pemphigus foliaceous is the most common autoimmune skin disease recognized in cats and most commonly presents as a pustular, varyingly erosive, and crusting dermatitis. (mspca.org)
  • Pemphigus foliaceous can make this tricky in the cat, as this type of disease commonly waxes and wanes, can be rapid or very slowly progressive, and spontaneously improve. (mspca.org)
  • Pemphigus vulgaris is a rare autoimmune disease and many health practitioners are still unaware of it. (deepdyve.com)
  • Pemphigus is a potentially fatal disease, associated with a mortality rate as high as 90-100 percent when untreated [ 1 ]. (cdlib.org)
  • Prednisone in doses varying from 40 to 400 mg has been used in the treatment of pemphigus, the higher doses being required for 5-10 weeks for disease control [ 2 ]. (cdlib.org)
  • Interestingly, plasma concentrations of interleukin (IL)-27 known to exert primarily regulatory functions were also elevated in active pemphigus patients, pointing to a rather inflammatory role of IL-27 in disease pathogenesis. (uni-marburg.de)
  • Pemphigus vulgaris is a potentially fatal autoimune blistering disease. (vgrd.org)
  • Feline pemphigus is an autoimmune disease in which a cat's immune system does not recognize part of its body as its own and creates an immune reaction attacking it. (animalwised.com)
  • Pemphigus in cats is a dermatological disease characterized by an acantholysis or detachment of each cell of the epidermis that creates vesicles within it. (animalwised.com)
  • Paraneoplastic pemphigus is a disease associated with an underlying cancer, generally of lymphoproliferative origin. (animalwised.com)
  • Marzano AV, Vezzoli P, Mariotti F, Boneschi V, Caputo R, Berti E. Paraneoplastic pemphigus associated with follicular dendritic cell sarcoma and Castleman disease. (medscape.com)
  • Koch LH, Layton CJ, Pilichowska M, Stadecker MJ, Barak O. Paraneoplastic Pemphigus and Castleman's Disease in the Setting of Herpes Simplex Virus Infection. (medscape.com)
  • Hi doctor , myself kavya from hyderabad .i have one doubt that Should I marry my boyfriend since I'm having an autoimmune disease pemphigus vulgaris? (drankireddy.com)
  • Pemphigus Vulgaris is not a contagious disease. (drankireddy.com)
  • Hailey-Hailey disease, or benign familial chronic pemphigus, is a rare autosomal dominant disease that can be difficult to diagnose due to wide variation in clinical features. (vohrawoundcare.com)
  • Canine paraneoplastic pemphigus is a rare disease associated with neoplastic diseases. (dogsis.com)
  • Pemphigus vulgaris (PV) is a life-threate- disorders and to shed a light on the role of ning blistering skin disease in which pa- mast cells in autoimmune diseases [ 7 ]. (who.int)
  • Efgartigimod alfa is under clinical development by Argenx and currently in Phase III for Pemphigus Vulgaris. (pharmaceutical-technology.com)
  • An open labeled clinical trial aimed at assessing the efficacy and safety of pulse intravenous cyclophosphamide with daily oral prednisolone in the treatment of pemphigus was carried out. (cdlib.org)
  • True (acantholytic) pemphigus can be diagnosed based on an assessment of clinical manifestations and results of cytology, histology and immunology tests. (vestnikdv.ru)
  • It is not as painful as pemphigus vulgaris, and is often mis-diagnosed as dermatitis or eczema . (wikidoc.org)
  • Pemphigus vulgaris - This begins with painful blisters in your mouth that do not itch. (utah.edu)
  • The major symptom of pemphigus vulgaris is the development of clear, soft, and painful (sometimes tender) blisters of various sizes. (msdmanuals.com)
  • The absence of this immunologic protection for such a long time poses potentially serious problems for pemphigus patients who become infected with SARS-CoV-2. (medscape.com)
  • It is the most common form of pemphigus that occurs with the use of penicillamine occurring in as many as 10% of patients taking the drug for prolonged periods. (thedoctorsdoctor.com)
  • Patients with paraneoplastic pemphigus display the presence of lesions which is a denominator among these patients but the characteristics of the lesions are different. (spts.org)
  • Patients with paraneoplastic pemphigus usually have a poor prediction of survival but only very limited chances of surviving has been on record. (spts.org)
  • Since then, many patients with paraneoplastic pemphigus have been reported, and patients previously believed to have other diseases have been retrospectively diagnosed. (medscape.com)
  • [ 3 ] There is an association between paraneoplastic pemphigus and HLA class II DRB I*03 and HLA-Cw*14 (in Chinese patients). (medscape.com)
  • summarize the increasing role of cellular immunity in paraneoplastic pemphigus, evidenced by lesional mononuclear cells and elevated IL-6 levels in sera from paraneoplastic pemphigus patients. (medscape.com)
  • Therefore, blood samples were obtained from 34 pemphigus patients, of which twelve clinically well characterized patients with no or minimal intake of immunosuppressive agents were selected for comprehensive analysis. (uni-marburg.de)
  • Concerning the APC compartment, a major finding was reduced circulating frequencies of both myeloid and plasmacytoid dendritic cells (mDC and pDC, respectively) in active pemphigus patients. (uni-marburg.de)
  • Similarly, active pemphigus patients displayed an upregulation of CCR2 on blood CD14+ monocytes, as well as elevated numbers of circulating CD16+ monocytes with expansion of the nonclassical subset. (uni-marburg.de)
  • However, functional analysis on the cellular level could not confirm an enhanced immunogenicity status of APC in active pemphigus patients, yet we found in these patients increased plasma levels of the proinflammatory tumor necrosis factor (TNF)-α. (uni-marburg.de)
  • Strikingly, in pemphigus patients, a significant correlation was not only found between serum IgG auto-ab titers and plasma levels of both IL-6 and TNF-α, but also between auto-ab titers and plasma concentrations of IL-27. (uni-marburg.de)
  • Finally, as a further major finding, we observed an increase of both Th17 cells and IL-10-secreting T cells in active pemphigus patients. (uni-marburg.de)
  • Hospital prevalence was 0.084% with a frequency of 9.1% among patients with pemphigus. (accessdermatology.com)
  • The International Pemphigus & Pemphigoid Foundation's most important objectives are to provide patients and doctors worldwide with information about pemphigus and pemphigoid. (pemphigus.org)
  • This will involve scoring pemphigus severity of patients at Premier Specialists using PDAI and IGA scores and a Likert score to classify patients as improved, stable or deteriorated compared to previous visits. (clinicaltrials.gov)
  • Genotyping of HLA-I and HLA-II alleles in Chinese patients with paraneoplastic pemphigus. (medscape.com)
  • Hertzberg MS, Schifter M, Sullivan J, Stapleton K. Paraneoplastic pemphigus in two patients with B-cell non-Hodgkin's lymphoma: significant responses to cyclophosphamide and prednisolone. (medscape.com)
  • In 1964, autoantibodies against keratinocyte surfaces were described in patients with pemphigus. (diseasesdic.com)
  • We aimed to determine the significance of detecting expression of some antigens that might be pivotal to the process, namely CD44 and CD117, in patients with active pemphigus vulgaris. (who.int)
  • Seventeen patients with ac- tive pemphigus vulgaris and 19 normal healthy controls were included in the study. (who.int)
  • In addition, there was a significant increase in a soluble form of c-kit in sera of patients with active pemphigus vulgaris compared to controls. (who.int)
  • Notre objectif était de déterminer l'importance de la détection de l'expression de certains antigènes pouvant jouer un rôle essentiel dans le processus, à savoir le CD44 et le CD117, chez des patients atteints de pemphigus vulgaire actif. (who.int)
  • Dix-sept patients atteints de pemphigus vulgaire actif et 19 témoins sains ont été inclus dans l'étude. (who.int)
  • Le pourcentage de CD44 dans les lymphocytes T périphériques était significativement plus élevé chez les patients que chez les témoins, comme détecté par la cytométrie en flux. (who.int)
  • En outre, il y avait une aug- mentation significative de la forme soluble du c-kit dans le sérum des patients atteints de pemphigus vulgaire actif par rapport aux témoins. (who.int)
  • This is a superficial form of pemphigus and accounts for 10% of all cases. (thedoctorsdoctor.com)
  • Desmosomal proteins are expressed to different degrees depending on the anatomical site, such as the footpads and periocular skin, which is most likely the cause for the more superficial presentation of pemphigus foliaceous. (mspca.org)
  • Pemphigus defines a group of autoimmune intraepithelial blistering diseases that are characterized by loss of normal cell-cell adhesion (acantholysis), and by the presence of pathogenic (predominantly IgG) autoantibodies reacting against epithelial adhesion molecules. (wikipedia.org)
  • Histopathologically, epidermal acantholysis and neutrophil infiltration predominate, hence the synonyms intraepidermal neutrophilic (IEN) IgA dermatosis, intraepidermal IgA pustulosis, IgA herpetiform pemphigus, and intercellular IgA vesiculopustular dermatosis have been used to describe this group of diseases. (medscape.com)
  • Unlike in the related pemphigoid , pemphigus manifests as intra-epithelial clefting, meaning the spinous cells of the epithelium break apart, a phenomenon known as acantholysis . (wikidoc.org)
  • Unlike pemphigus vulgaris, it usually only affects the skin, with the appearance of lesions at the mucocutaneous junctions or in the oral cavity being rare. (dogsis.com)
  • Pemphigus is derived from the Greek pemphix , meaning bubble or blister. (wikidoc.org)
  • Pemphigus-like": causing blister crust over the raw exuding skin lesion. (spts.org)
  • The primary lesion of pemphigus vulgaris is a soft blister filled with clear fluid that appears on healthy or irritated skin. (diseasesdic.com)
  • The pemphigus foliaceous variant of the pemphigus complex is caused by autoantibodies targeting a specific component of keratinocyte desmosomes. (mspca.org)
  • Studies have confirmed the role of anti-keratinocyte IgG in cats with pemphigus foliaceous. (mspca.org)
  • Acantholytic keratinocytes are reported in 74% of cats with pemphigus foliaceous. (mspca.org)
  • However, the presence of acantholytic keratinocytes is not singularly pathognomonic for pemphigus foliaceous (certain strains of Staphylococcus pseudintermedius and Trichophyton spp. (mspca.org)
  • If lesions persist while the bacterial component resolves, this is a stronger indication of a likely underlying pemphigus foliaceous. (mspca.org)
  • Pemphigus foliaceous (PF) is undeniably the most common pemphigus variety worldwide. (vin.com)
  • The foliaceous pemphigus it can start with the administration of certain drugs. (dogsis.com)
  • With pemphigus vulgaris, the immune system looks for proteins that bind the cells of the skin. (hopkinsmedicine.org)
  • Pemphigus causes the immune system to fight against the body's own cells in the same way that it fights off invading germs. (hopkinsmedicine.org)
  • With pemphigus vulgaris, the immune system starts to fight against the normal proteins that bind the cells of the skin together. (franciscanhealth.org)
  • Pemphigus vulgaris occurs when the immune system mistakenly attacks proteins in the upper layers of the skin. (msdmanuals.com)
  • Body-wide (systemic) therapy is needed to control pemphigus and should be started as early as possible. (medlineplus.gov)
  • If not treated, pemphigus is fatal, due to overwhelming systemic infection . (wikidoc.org)
  • Paraneoplastic pemphigus associated with systemic mastocytosis. (medscape.com)
  • 2 What causes cause pemphigus in dogs? (dogsis.com)
  • 5-Point scale from 0-4 to score the severity of pemphigus , where 0=clear, 1=almost clear, 2=mild, 3=moderate and 4=severe. (clinicaltrials.gov)
  • In these cases, tumor removal may lead to a remission of the pemphigus. (wikidoc.org)
  • estudio documental, realizado de mayo a julio de 2022, en páginas web de instituciones de educación superior que ofrecieron estos cursos. (bvsalud.org)
  • Unlike pemphigus, oral mucosal involvement is rare. (thedoctorsdoctor.com)
  • In the first part of the study, 8 dermatologists will score 20 sets of photographs of pemphigus lesions (a mixture of skin and mucosal lesions), using the IGAs for pemphigus as well as the PDAI and ABSIS. (clinicaltrials.gov)
  • Kennedy NA, Dawe S. Atypical paraneoplastic pemphigus secondary to endometrial carcinoma with no mucosal involvement. (medscape.com)
  • Direct immunofluorescence microscopy performed on epithelial biopsy specimen obtained from a patient with pemphigus vulgaris detects immunoglobulin G deposits at the epithelial cell surfaces. (medscape.com)
  • The endemic form of Pemphigus stimulates the curiosity of Brazilian dermatologists mainly because of its possible occurrence in animals raised up in Brazilian conditions as well as in susceptible individuals, exposed to a huge number of arthropods, such as aboriginal individuals, farmers and workers living in miserable social, unhygienic conditions and in close contact with insects. (vin.com)
  • Two subtypes of IgA pemphigus have been identified: subcorneal pustular dermatosis (SPD)-type IgA pemphigus and IEN-type IgA dermatosis. (medscape.com)
  • Although pemphigus vulgaris may occur at any age, it is quite rare in children, and most common in the middle aged and elderly. (wikidoc.org)
  • Paraneoplastic pemphigus - This is a rare form of pemphigus that can occur in people with certain types of cancer. (utah.edu)
  • Pemphigus can occur at any age, but it's most often seen in people who are middle-aged or older. (ahdubai.com)
  • Blisters occur with a number of more common conditions, so pemphigus, which is rare, can be difficult to diagnose. (ahdubai.com)
  • Immunoglobulin A (IgA) pemphigus is a group of newly characterized immune-mediated intraepidermal blistering skin diseases. (medscape.com)
  • Other skin diseases besides PF also have the word "pemphigus" in their name, but are different from PF. (vin.com)
  • The canine pemphigus complex encompasses a group of autoimmune skin diseases, which are characterized by loss of cohesion between skin cells . (dogsis.com)