Paraneoplastic pemphigusVulgarisFoliaceusDesmogleinAutoimmuneAntibodiesEpithelialSystemicPulmonaryConstrictivePemphixSyndromeTissueDiagnosisFatalOccursLungOverviewFormsTreatmentDiseaseCellsLeadAssociated with paraneoplastic pemphigusConstrictive bronchiolitisParaneoplastic autoimmune multiorgan syndromeAutoantibodiesAutoantibody-mediatedHerpetiformisBenignSeverityAntigenSubtypesVariantMucocutaneousTumorBlistering and raw soresSevereMucosalDermatosisSymptomsComplicationRespiratoryErosionsAffectsPatientsRareCancerSkinTypeChild
Paraneoplastic pemphigus13
- [ 1 ] first described paraneoplastic pemphigus in 1990. (medscape.com)
- Since then, many patients with paraneoplastic pemphigus have been reported, and patients previously believed to have other diseases have been retrospectively diagnosed. (medscape.com)
- Paraneoplastic pemphigus is rare, and, while the exact incidence is unknown, it is likely underreported. (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)
- Hematologic malignancies are most commonly associated with paraneoplastic pemphigus, although it can also be associated with carcinomas, sarcomas, and benign neoplasms. (medscape.com)
- Unlike other forms of pemphigus, paraneoplastic pemphigus affects organ systems other than the integument. (medscape.com)
- Paraneoplastic pemphigus is an autoimmune disorder initiated by an underlying neoplasm. (medscape.com)
- Passive transfer of paraneoplastic pemphigus sera causes blistering in neonatal mice, proving that the antibodies present are pathogenic. (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)
- The several types of pemphigus (pemphigus vulgaris, pemphigus foliaceus, intraepidermal neutrophilic IgA dermatosis, and paraneoplastic pemphigus) vary in severity. (wikipedia.org)
- The least common and most severe type of pemphigus is paraneoplastic pemphigus (PNP). (wikipedia.org)
- There are three types of pemphigus which vary in severity: pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus. (wikidoc.org)
- With Intravenous Immunoglobulin (IVIG) treatment and aggressive chemotherapy for the underlying lympoma, there are few known survivors of Paraneoplastic Pemphigus with pulmonary involvement (contact information in External Links below). (wikidoc.org)
Vulgaris10
- 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)
- 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)
- 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 further divided in two major subtypes: pemphigus vulgaris (PV) and pemphigus foliaceus (PF). (wikipedia.org)
- Pemphigus Vulgaris. (wikidoc.org)
- The most common form of the disorder is pemphigus vulgaris ( ICD-10 L10.0). (wikidoc.org)
- 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)
- Pemphigus vulgaris often affects people between the ages of 40-60, and mainly of Jewish, Middle Eastern, or Mediterranean descent. (wikidoc.org)
- It is not as painful as pemphigus vulgaris, and is often mis-diagnosed as dermatitis or eczema . (wikidoc.org)
- 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)
Foliaceus4
- 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)
- 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)
- Pemphigus foliaceus is characterized by crusty sores that often begin on the scalp , and may move to the chest, back, and face. (wikidoc.org)
Desmoglein1
- In pemphigus, autoantibodies form against desmoglein, which forms the "glue" that attaches adjacent epidermal cells via attachment points called desmosomes. (wikipedia.org)
Autoimmune4
- Pemphigus (/ˈpɛmfɪɡəs/ or /pɛmˈfaɪɡəs/) is a rare group of blistering autoimmune diseases that affect the skin and mucous membranes. (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)
- 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)
- Pemphigus is an autoimmune disorder that causes blistering and raw sores on skin and mucous membranes . (wikidoc.org)
Antibodies2
- 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)
- The presence of these antibodies indicates pemphigus. (wikidoc.org)
Epithelial1
- 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)
Systemic1
- If not treated, pemphigus is fatal, due to overwhelming systemic infection . (wikidoc.org)
Pulmonary1
- Bronchiolitis in asthma and chronic obstructive pulmonary disease. (pneumon.org)
Constrictive1
- In this variety of pemphigus, the disease process often involves the lungs, causing bronchiolitis obliterans (constrictive bronchiolitis). (wikipedia.org)
Pemphix1
- Pemphigus is derived from the Greek pemphix , meaning bubble or blister. (wikidoc.org)
Syndrome1
- Bronchiolitis obliterans syndrome 2001: an update of the diagnostic criteria. (pneumon.org)
Tissue3
- In this variety of pemphigus, the disease process often involves Bronchiolitis obliterans , a fatal destruction of alveoli in lung tissue. (wikidoc.org)
- Bronchiolitis is a generic term applied to a variety of inflammatory diseases that affect the bronchioles, sparing a considerable portion of the other parenchymal structures, in which both inflammatory cells and mesenchymal tissue are present. (pneumon.org)
- Bronchiolitis in association with connective tissue disorders. (pneumon.org)
Diagnosis1
- A diagnosis of neoplastic pemphigus may prompt a search for an existing tumor. (wikidoc.org)
Fatal1
- Skin lesions caused by pemphigus can lead to fatal infections, so treatment is extremely important. (wikipedia.org)
Occurs1
- Bronchiolitis occurs in a variety of clinical settings and may be associated with large airway disease and parenchymal disease. (pneumon.org)
Lung1
- Bronchiolitis obliterans after human lung transplantation. (pneumon.org)
Overview1
- King TE Jr. Overview of bronchiolitis. (pneumon.org)
Forms1
- 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)
Treatment1
- Currently many clinical trials are in progress aimed at the identification of the most appropriate treatment for each specific type of bronchiolitis. (pneumon.org)
Disease2
- 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)
- HRCT of obliterative bronchiolitis and other small airways disease. (pneumon.org)
Cells2
- A biopsy of a suspected lesion is taken: a sample of the blistered skin is removed and examined under the microscope to determine if the cells are separated in the manner characteristic of pemphigus. (wikidoc.org)
- Also apparent in pemphigus is a "tombstone appearance" of the basal cell layer and Tzanck cells. (wikidoc.org)
Lead1
- In these cases, tumor removal may lead to a remission of the pemphigus. (wikidoc.org)
Associated with paraneoplastic pemphigus2
- Hematologic malignancies are most commonly associated with paraneoplastic pemphigus, although it can also be associated with carcinomas, sarcomas, and benign neoplasms. (medscape.com)
- 4. [Bronchiolitis obliterans associated with paraneoplastic pemphigus: a paraneoplastic autoimmune multiorgan syndrome]. (nih.gov)
Constrictive bronchiolitis4
- In this variety of pemphigus, the disease process often involves the lungs, causing bronchiolitis obliterans (constrictive bronchiolitis). (wikipedia.org)
- 2. Constrictive bronchiolitis obliterans and paraneoplastic pemphigus. (nih.gov)
- 7. Paraneoplastic pemphigus with constrictive bronchiolitis obliterans. (nih.gov)
- 8. Constrictive bronchiolitis associated with paraneoplastic autoimmune multi-organ syndrome. (nih.gov)
Paraneoplastic autoimmune multiorgan syndrome2
Autoantibodies6
- In pemphigus, autoantibodies form against desmoglein, which forms the "glue" that attaches adjacent epidermal cells via attachment points called desmosomes. (wikipedia.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)
- Paraneoplastic pemphigus is caused by production of autoantibodies against various epidermal proteins involved in cell adhesion. (bvsalud.org)
- Background: Patients with paraneoplastic pemphigus (PNP) and inflammatory bowel diseases, such as Crohn's disease (CD) and ulcerative colitis (UC), develop autoantibodies against simple epithelial cells. (elsevierpure.com)
- Conclusion: These findings indicate that IgG autoantibodies against Dsg2 are not involved in PNP, CD or UC and suggest the existence of other unknown cell surface target antigen(s) in bronchiolitis obliterans in PNP. (elsevierpure.com)
- In Pemphigus, autoantibodies attack proteins that connect each epidermal cell to each other. (psderm.com)
Autoantibody-mediated2
- Paraneoplastic pemphigus (PNP) is an autoantibody-mediated mucocutaneous blistering disease associated with underlying neoplasms. (the-medical-dictionary.com)
- About 20-30% of patients with PNP develop fatal bronchiolitis obliterans, in which autoantibody-mediated injury is suspected because of in vivo IgG deposition on cell surfaces of bronchial epithelia. (elsevierpure.com)
Herpetiformis2
- Pemphigus Herpetiformis Pemphigus herpetiformis is considered to be a distinct entity. (medscape.com)
- IgA Pemphigus Clinically, IgA pemphigus presents annular erythemas with minimal blisters or vesicles arranged at the periphery, mimicking pemphigus herpetiformis or dermatitis herpetiformis. (medscape.com)
Benign2
- 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)
- Paraneoplastic pemphigus is a rare and severe autoimmune blistering disease characterized by a recalcitrant and severe mucositis, and polymorphic cutaneous lesions, associated with benign and malignant neoplasms. (bvsalud.org)
Severity1
- Severity is assessed by the pemphigus disease activity index (PDAI). (medscape.com)
Antigen1
- [ 14 ] The antigen of SPD-type IgA pemphigus is desmocollin-1, while the antigen of IEN-type IgA pemphigus is still unknown. (medscape.com)
Subtypes1
- [ 27 ] There are two subtypes for pemphigus vegetans. (medscape.com)
Variant1
Mucocutaneous1
- Abstract Paraneoplastic pemphigus (PNP) is a rare autoimmune mucocutaneous blistering disease that is commonly associated with underlying B-cell neoplasms. (the-medical-dictionary.com)
Tumor2
- 9. Paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis and bronchiolitis obliterans. (nih.gov)
- In these cases, tumor removal may lead to a remission of the pemphigus. (the-medical-dictionary.com)
Blistering and raw sores2
- Pemphigus is an autoimmune disorder that causes blistering and raw sores on skin and mucous membranes. (the-medical-dictionary.com)
- Pemphigus presents as blistering and raw sores inside the mouth or on the skin. (psderm.com)
Severe4
- The least common and most severe type of pemphigus is paraneoplastic pemphigus (PNP). (wikipedia.org)
- A child with severe bronchiolitis may get fatigued from the work of breathing and have poor air movement in and out of the lungs due to the clogging of the small airways. (symptoma.com)
- Two months ago, the patient was seen for tachypnea and wheezing, and diagnosed with severe respiratory syncytial virus (RSV) bronchiolitis. (symptoma.com)
- Pemphigus can cause scarring in severe cases, or when secondary infections occur. (psderm.com)
Mucosal1
- Paraneoplastic Pemphigus Paraneoplastic pemphigus classically presents intractable oral mucosal lesions and pseudomembranous conjunctivitis. (medscape.com)
Dermatosis1
- [ 14 ] IgA pemphigus is subdivided into subcorneal pustular dermatosis (SPD)-type and intraepidermal neutrophilic IgA dermatosis (IEN)-type. (medscape.com)
Symptoms3
- Issues to be addressed when evaluating a patient for pemphigus include the age of onset, previous clinical symtoms, initial presentation, presence of mucous membrane symptoms and the presence of Nikolsky's phenomenon. (medscape.com)
- In the preliminary stages, symptoms of bronchiolitis are similar to those of common cold . (symptoma.com)
- The early symptoms of bronchiolitis are similar to those of a common cold, such as a runny nose and a cough . (symptoma.com)
Complication1
- Bronchiolitis obliterans (BO) is a frequent complication which contributes to the high mortality rate of PNP/PAMS. (nih.gov)
Erosions2
- Neumann-type pemphigus vegetans initially presents with bullae and erosions, which then become proliferative. (medscape.com)
- [ 30 ] The skin lesions of paraneoplastic pemphigus are variable, including erythemas, flaccid bullae, tense bullae, erosions, erythema multiforme-like skin lesions and lichen planus-like skin lesions. (medscape.com)
Affects1
- Unlike other forms of pemphigus, paraneoplastic pemphigus affects organ systems other than the integument. (medscape.com)
Patients5
- 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)
- Bronchiolitis obliterans (BO) is one of the leading causes of mortality in these patients. (bvsalud.org)
- Our findings support the limited role of radiography in the evaluation of hospitalized infants with bronchiolitis, especially patients without fever or hypoxia. (symptoma.com)
Rare2
- Paraneoplastic pemphigus is rare, and, while the exact incidence is unknown, it is likely underreported. (medscape.com)
- Pemphigus (/ˈpɛmfɪɡəs/ or /pɛmˈfaɪɡəs/) is a rare group of blistering autoimmune diseases that affect the skin and mucous membranes. (wikipedia.org)
Cancer1
- This type of pemphigus is associated with underlying cancer. (psderm.com)
Skin1
- 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)
Type2
- Hallopeau-type pemphigus vegetans starts with pustules on the intertriginous regions, and after that it develops proliferative lesions. (medscape.com)
- [ 28 ] Neumann-type pemphigus vegetans is more proliferative and intractable than Hallopeau-type pemphigus vegetans. (medscape.com)
Child1
- 3. Paraneoplastic pemphigus with bronchiolitis obliterans in a child. (nih.gov)