Senear-Usher syndrome or pemphigus erythematosus is a pathology that overlaps clinically and serologically with pemphigus foliaceus and lupus erythematosus. Skin biopsies of patients with pemphigus erythematosus reveal acantholysis and deposits of immunoglobulins in desmosomes, and they are positive in the lupus band test. In the present paper, we determined whether the autoantibodies associated with pemphigus erythematosus targeted a single antigen or multiple antigens as a result of the stimulation of independent B cell clones. Our present paper demonstrates that patients with pemphigus erythematosus produce both antiepithelial antibodies specific for desmoglein 1 and 3 and antinuclear antibodies specific for Ro, La, Sm, and double-stranded DNA antigens. After eluting specific anti-epithelial or anti-nuclear antibodies, which were recovered and tested using double-fluorescence assays, a lack of cross-reactivity was demonstrated between desmosomes and nuclear and cytoplasmic lupus antigens. ...
Risks for Pemphigus foliaceus, Pemphigus foliaceus treatments, recommended products for Pemphigus foliaceus, ways to prevent Pemphigus foliaceus, causes of Pemphigus foliaceus
TY - JOUR. T1 - Immunologic and histopathologic characterization of an active disease mouse model for pemphigus vulgaris. AU - Ohyama, Manabu. AU - Amagai, Masayuki. AU - Tsunoda, Kazuyuki. AU - Ota, Takayuki. AU - Koyasu, Shigeo. AU - Hata, Jun Ichi. AU - Umezawa, Akihiro. AU - Nishikawa, Takeji. N1 - Copyright: Copyright 2017 Elsevier B.V., All rights reserved.. PY - 2002. Y1 - 2002. N2 - Pemphigus vulgaris is an autoimmune blistering disease of the skin and mucous membranes that is caused by anti-desmoglein 3 IgG autoantibodies. Recently, we generated an active disease mouse model for pemphigus vulgaris by adoptive transfer of splenocytes from immunized desmoglein 3-/- mice to Rag2-/- mice. In this study, we performed immunologic and histopathologic studies using this pemphigus vulgaris model in mice and compared the gross and microscopic phenotypes of pemphigus vulgaris model mice and desmoglein 3-/- mice. Pemphigus vulgaris model mice showed strong in vivo IgG, and weak IgA deposition on ...
Define pemphigus vulgaris. pemphigus vulgaris synonyms, pemphigus vulgaris pronunciation, pemphigus vulgaris translation, English dictionary definition of pemphigus vulgaris. n. Any of several acute or chronic skin diseases characterized by groups of itching blisters. pem′phi·gous adj. n pathol any of a group of blistering skin...
TY - JOUR. T1 - Neonatal pemphigus vulgaris. T2 - IgG4 autoantibodies to desmoglein 3 induce skin blisters in newborns. AU - Parlowsky, Thomas. AU - Welzel, Julia. AU - Amagai, Masayuki. AU - Zillikens, Detlef. AU - Wygold, Thorsten. PY - 2003/4/1. Y1 - 2003/4/1. N2 - We report a case of neonatal pemphigus vulgaris presenting with skin lesions on the head, genital area, and right foot. Pemphigus vulgaris was diagnosed by the presence of circulating autoantibodies predominantly of the IgG4 subtype by indirect immunofluorescence microscopy and by enzyme-linked immunosorbent assay using recombinant desmoglein 3. This case demonstrates the pathogenic relevance of IgG4 autoantibodies to desmoglein 3 in the skin of neonates.. AB - We report a case of neonatal pemphigus vulgaris presenting with skin lesions on the head, genital area, and right foot. Pemphigus vulgaris was diagnosed by the presence of circulating autoantibodies predominantly of the IgG4 subtype by indirect immunofluorescence microscopy ...
We evaluated the effectiveness of mizoribine, a newly developed immunosuppressive agent, as an adjuvant therapy in the treatment of both pemphigus vulgaris and pemphigus foliaceus. Eleven pemphigus patients (eight pemphigus vulgaris and three pemphigus foliaceus) received the combination therapy of prednisolone and mizoribine. Complete remission was observed in three of the eight patients with pemphigus vulgaris and in one of the three patients with pemphigus foliaceus. The four patients with complete remission had a rapid clinical response and achieved remission at a median of 11.8 months. Partial remission was achieved in two of the three patients with pemphigus foliaceus. The median time to achieve partial remission was 16.0 months. Six (55.6%) of the 11 patients with pemphigus had complete or partial remission and were able to taper their prednisolone. The cumulative probability of having a complete remission was 64.3% at 19 months of follow-up using Kaplan-Meier analysis. The effectiveness ...
This page includes the following topics and synonyms: Pemphigus Foliaceus, Superficial Pemphigus, Pemphigus erythematosus, Senear-Usher Syndrome, Fogo selvagem, Pemphigus herpetiformis.
Background Paraneoplastic pemphigus (PNP) is a devastating autoimmune blistering disease, involving mucocutaneous and internal organs, and associated with underlying neoplasms. PNP is characterized by the production of autoantibodies targeting proteins of the plakin and cadherin families involved in maintenance of cell architecture and tissue cohesion. Nevertheless, the identity of an antigen of Mr 170,000 (p170), thought to be critical in PNP pathogenesis, has remained unknown. Methodology/Principal Findings Using an immunoprecipitation and mass spectrometry based approach, we identified p170 as alpha-2-macroglobuline-like-1, a broad range protease inhibitor expressed in stratified epithelia and other tissues damaged in the PNP disease course. We demonstrate that 10 PNP sera recognize alpha-2-macroglobuline-like-1 (A2ML1), while none of the control sera obtained from patients with bullous pemphigoid, pemphigus vulgaris, pemphigus foliaceus and normal subjects does. Conclusions/Significance Our study
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Pemphigus is a group of five autoimmune skin diseases characterized by vesicles and bullae (large and small blisters) in the mouth and at mucocutaneous junctions (the junctions between skin and mucosal tissues). Commonly affected areas include the eyelids, lips, nostrils, and anus.. Pemphigus Foliaceus (PF) - The term means leaf-like pemphigus. This is the most common autoimmune skin disease of cats. With PM, the patient develops crusts (scabs) and ulcers around the eyes, ears, footpads, groin, and bridge of the nose. In cats, lesions also develop at the toenail beds creating crusty sore feet. PM is rarely found in the mouth or at mucocutaneous junctions. It usually appears suddenly without a recognized cause, but in some cases it may be drug induced or can be the result of years of chronic skin disease.. Pemphigus Vulgaris (PV) - The term means common pemphigus and it is the most frequent form of pemphigus in humans. Fluid filled blisters called vesicles form in and around the mouth, ...
Pemphigus foliaceus is one of the most common autoimmune diseases in dogs, leading to autoantibodies to desmoglein I and finally to pustule and crust formation. Immunosuppressive therapy is mandatory in this disease - but does it always have to be given for the rest of the life? An interesting, rarely discussed question!
Senear-Usher syndrome or pemphigus erythematosus is a pathology that overlaps clinically and serologically with pemphigus foliaceus and lupus erythematosus. Skin biopsies of patients with pemphigus erythematosus reveal acantholysis and deposits of immunoglobulins in desmosomes, and they are positive in the lupus band test. In the present paper, we determined whether the autoantibodies associated with pemphigus erythematosus targeted a single antigen or multiple antigens as a result of the stimulation of independent B cell clones. Our present paper demonstrates that patients with pemphigus erythematosus produce both antiepithelial antibodies specific for desmoglein 1 and 3 and antinuclear antibodies specific for Ro, La, Sm, and double-stranded DNA antigens. After eluting specific anti-epithelial or anti-nuclear antibodies, which were recovered and tested using double-fluorescence assays, a lack of cross-reactivity was demonstrated between desmosomes and nuclear and cytoplasmic lupus antigens. This result
Kirov State Medical University. The article describes modern views on predisposing factors, characteristics of the immunological changes and link with the histocompatibility antigens HLA, the role of circulating autoantibodies in the pathogenesis of the disease in patients with pemphigus vulgaris. Described 2 cases of clinical observations and vulgar pemphigus, foliaceous pemphigus, accompanied by concomitant pathology of internal organs in the form of cardiovascular system pathology, chronic iron deficiency anemia, diffuse and knotty changes of the thyroid gland. The good effect of etiotropic, pathogenetic and symptomatic therapy in accordance with the standards of specialized medical care in moderate-to-severe pemphigus. ...
Indication: adult patients with pemphigus vulgaris and pemphigus foliaceus.. SAMEP recommendation: To list rituximab on the statewide high cost medicines formulary for adult patients with pemphigus vulgaris and pemphigus foliaceus who are refractory to conventional immunosuppression or who are steroid-dependent.. Rationale: Rituximab, in combination with short term prednisone, can induce remission in newly diagnosed pemphigus patients with a lower total prednisone dose than if relying on prednisone alone. The majority of patients treated with rituximab experienced at least one episode of complete remission off therapy (89%) at two years as compared to approximately a third (34%) of patients only receiving prednisone.. The rate of relapse at 24 months also favoured rituximab treated patients (24% versus 45%) and fewer patients in the rituximab arm had active lesions at 24 months or had no lesions but remained on significant prednisone doses (11% versus 64%). Rituximab has a steroid sparing effect ...
The purpose of this study is to explore a new medication for the skin disease termed pemphigus. Our specific aim is to determine whether the use of sirolimus will allow for a decrease in the dosage or possibly eliminate the need for corticosteroids, which so far is the only type of drug that can control this disease.. Pemphigus is an autoimmune disease characterized by blistering, caused by autoantibodies against certain cells in the skin. This disease most commonly occurs in individuals ages 50 and older, and it presents as painful shallow erosions and/or blisters in the mouth and/or skin. Pemphigus is very painful and uncomfortable, associated with impaired quality of life and significant morbidity. Severe or untreated cases of pemphigus can become fatal if the involved surface area becomes large enough to cause dehydration and/or infection. The first line of therapy, and the standard of care, for pemphigus remain to be systemic corticosteroids. However, corticosteroids have many known side ...
The purpose of this study is to explore a new medication for the skin disease termed pemphigus. Our specific aim is to determine whether the use of sirolimus will allow for a decrease in the dosage or possibly eliminate the need for corticosteroids, which so far is the only type of drug that can control this disease.. Pemphigus is an autoimmune disease characterized by blistering, caused by autoantibodies against certain cells in the skin. This disease most commonly occurs in individuals ages 50 and older, and it presents as painful shallow erosions and/or blisters in the mouth and/or skin. Pemphigus is very painful and uncomfortable, associated with impaired quality of life and significant morbidity. Severe or untreated cases of pemphigus can become fatal if the involved surface area becomes large enough to cause dehydration and/or infection. The first line of therapy, and the standard of care, for pemphigus remain to be systemic corticosteroids. However, corticosteroids have many known side ...
In multivariate survey logistic regression models adjusting for age, sex and race/ethnicity, pemphigus and its treatments were associated with 39 of 122 comorbidities examined. The disorders most strongly associated with pemphigus were Cushing syndrome [adjusted odds ratio (OR) 17·23, 95% confidence interval (CI) 2·41-122·90], adrenal insufficiency (4·08, 1·71-9·73), myasthenia gravis (6·92, 2·55-18·79), mucositis (17·19, 7·73-38·22), herpes infection (7·98, 3·62-17·62), fungal infections (4·03, 3·60-4·52), insomnia (18·02, 2·46-131·88) and hidradenitis (5·34, 1·33-21·43). Among malignancies, only leukaemia (OR 1·56, 95% CI 1·08-2·24) and non-Hodgkin lymphoma (1·52, 1·15-2·03) were associated with pemphigus, but not any solid organ malignancies. Patients with a secondary diagnosis of pemphigus had higher inpatient mortality (3·20%, 95% CI 2·71-3·69) than those with a primary (1·60%, 1·29-1·91) or no (1·78%, 1·78-1·78) diagnosis of pemphigus (P , 0·001 ...
Background:Pemphigus is a life-threatening, autoimmune blistering disease, mediated by IgG autoantibodies. The aim of our study was to assess the usefulness of a new enzyme-linked immunosorbent assay (ELISA) in detecting circulating pemphigus autoantibodies, and to compare its sensitivity and specificity with the indirect immunofluorescence (IIF) test. We also established the frequency of occurrence of pemphigus autoantibodies in relatives of our patients.Material/Methods:IIF and ELISA tests were performed in 24 patients with pemphigus vulgaris, 13 with pemphigus foliaceus, 56 healthy relatives, and 50 controls, selected according to sex and age.Results:The obtained results revealed high specificity and sensitivity of ELISA, comparable to the IIF test, especially in patients who were in the active stage of the disease. We also showed that the profile of anti-Dsg 1 and/or anti-Dsg 3 autoantibodies is associated with the clinical variant of pemphigus. The frequency of occurrence of pemphigus ...
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The rest of December went well, eye healing up and skin was getting better. After the surgery Otis started to gain weight and muscle. I think the eye was bothering him for much longer than we knew. Somewhere in the back of my mind I crossed my fingers that the tumor had caused all of…
In patients with pemphigus vulgaris (PV), autoantibodies against desmoglein 3 (Dsg3) cause loss of cell-cell adhesion of keratinocytes in the basal and immediate suprabasal layers of stratified squamous epithelia. The pathology, at least partially, may depend on protease release from keratinocytes, but might also result from antibodies interfering with an adhesion function of Dsg3. However, a direct role of desmogleins in cell adhesion has not been shown. To test whether Dsg3 mediates adhesion, we genetically engineered mice with a targeted disruption of the DSG3 gene. DSG3 -/- mice had no DSG3 mRNA by RNase protection assay and no Dsg3 protein by immunofluorescence (IF) and immunoblots. These mice were normal at birth, but by 8-10 d weighed less than DSG3 +/- or +/+ littermates, and at around day 18 were grossly runted. We speculated that oral lesions (typical in PV patients) might be inhibiting food intake, causing this runting. Indeed, oropharyngeal biopsies showed erosions with histology
Pemphigus foliaceus is a rare autoimmune disorder that causes people to have skin that blisters easily and develops crusty, scaly...
Effectiveness and side effects of anti-CD20 therapy for autoantibody-mediated blistering skin diseases: A comprehensive survey of 71 consecutive patients from the Initial use to 2007 Jennifer D Peterson1, Lawrence S Chan2,3,41Department of Dermatology, Texas Tech University Health Sciences Center at Lubbock, Lubbock, TX, USA; 2Department of Dermatology; 3Department of Microbiology/Immunology, University of Illinois at Chicago, Chicago, IL, USA; 4Medicine Service, Jesse Brown VA Medical Center, Chicago, IL, USAAbstract: In order to examine the efficacy and side effects of the monoclonal antibody anti-CD20 (rituximab) on autoimmune blistering skin diseases, we performed a comprehensive survey of 71 consecutive patients from initial use up to 2007, using the PubMed database. A heterogeneous group of patients, including 51 patients with pemphigus vulgaris, one with pemphigus vegetans, nine with pemphigus foliaceus, five with paraneoplastic pemphigus, four with epidermolysis bullosa acquisita, and one with
A prospective study of laboratory findings in dapsone treated patients with Pemphigus vulgaris and foliaceus, Mona Afrasiabi, Shabnam Esmaeilinejad, Vahid Asgary, Reza Ahangari Coh
Pemphigus describes a group of autoimmune blistering diseases that involve the epidermal surfaces of the skin, mucosa, or both. There are 3 broad categories: pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP). Pemphigus is mediated by an autoantibody that binds a specific component of the desmosomal plaque. The desmosomal plaque holds the keratinocytes of the skin and mucosa together. ...
Background: The use of saliva for the diagnosis of pemphigus vulgaris (PV) by enzyme-linked immunosorbent assay (ELISA) using desmoglein (Dsg)3 antigen has not been extensively documented, nor has the detection of serum IgA antibodies to Dsg3. Objectives: (i) To establish whether whole saliva might provide a suitable alternative to serum for diagnosing and monitoring PV; (ii) to investigate whether anti-Dsg3 IgA antibodies can be detected in serum and saliva and (iii) to establish whether there is an association between serum or saliva anti-Dsg3 antibodies and disease severity. Methods: Precoated Dsg3 ELISA plates were used to test serum and/or saliva for IgG and IgA antibodies. Matched serum and whole saliva samples were collected from 23 patients with PV, 17 healthy subjects and 19 disease controls. All patients with PV, disease controls and six healthy controls provided matched parotid saliva. Results: Whole saliva IgG antibodies to Dsg3 were detected in 14 of 23 patients (61%) and serum IgG ...
Formalin pigment shares many properties with hemozoin. In this case, the authors recommend the use of neutral buffered formalin to prevent the formation of formalin pigment especially after long periods of fixation when taking biopsies under extreme temperature and environmental humidity.
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Autoimmune bullous diseases (ABDs) are uncommon but significant skin disorders with relatively high morbidity and mortality. Some surveys have been carried out to describe the spectrum of ABDs in a region, but this is the first that has focused on ABDs in elderly patients. This study was conducted to determine the clinicoepidemiologic features of ABDs in elderly patients. Medical records of all ABD patients with disease onset after the age of 60 years who presented to the Autoimmune Bullous Diseases Research Center, Tehran, Iran between April 2003 and March 2013 were reviewed ...
Tong, J.C.,Tan, T.W.,Ranganathan, S.,Sinha, A.A. (2006). Prediction of desmoglein-3 peptides reveals multiple shared T-cell epitopes in HLA DR4- and DR6- associated Pemphigus vulgaris. BMC Bioinformatics 7 (SUPPL.5). ScholarBank@NUS Repository. https://doi.org/10.1186/1471-2105-7-S5-S7 ...
TY - JOUR. T1 - (3) Calcium enhances the sensitivity of immunofluorescence for pemphigus antibodies. AU - Matis, W. L.. AU - Anhalt, Grant James. AU - Diaz, L. A.. AU - Rivitti, E. A.. AU - Martins, C. R.. AU - Berger, Robert. PY - 1988. Y1 - 1988. UR - http://www.scopus.com/inward/record.url?scp=84988103834&partnerID=8YFLogxK. UR - http://www.scopus.com/inward/citedby.url?scp=84988103834&partnerID=8YFLogxK. U2 - 10.1111/j.1365-2133.1988.tb05377.x. DO - 10.1111/j.1365-2133.1988.tb05377.x. M3 - Article. AN - SCOPUS:84988103834. VL - 119. SP - 42. JO - British Journal of Dermatology. JF - British Journal of Dermatology. SN - 0007-0963. ER - ...
Pemphigus Vulgaris is mainly characterized by a formation of blisters in the mouth which make it difficult to swallow food or drink water. These blisters later
Corneal involvement is a rare complication in patients with pemphigus vulgaris. Severe corneal involvement has never been reported except in the case of a 56 year old man with severe ocular involvement including conjunctivitis, corneal ulceration, and perforation despite immunosuppressive therapy.4 Although a causative organism was not isolated, the authors suggested that the complications were due to an infectious agent.. Two mechanisms have been suggested to cause the corneal erosion-bacteria or other pathogenic organisms that infect the cornea because of the epithelial defect and tear film disorder brought on by the corticosteroid and immunosuppressive therapy. Although the culture obtained from right ocular discharge before starting ofloxacin ointment showed a negative result, we could not deny the bacterial infection. We did not perform a bacterial or viral culture or polymerase chain reaction examinations using a corneal sample.. The second mechanism is an autoimmune mechanism against one ...
TY - CHAP. T1 - Paraneoplastic pemphigus. AU - Diercks, G.F.H.. AU - Pas, Hendrikus. AU - Jonkman, Marcel F.. AU - Poot, Angelique. PY - 2016. Y1 - 2016. N2 - Paraneoplastic pemphigus is a rare but severe autoimmune disease characterized by severe stomatitis and a variety of cutaneous manifestations in association with an underlying neoplasia. Pulmonary involvement may also occur. The pathogenesis involves the production of autoantibodies against desmogleins, plakins, and the protease inhibitor alpha-2-macroglobulin-like 1, but T-cell-mediated autoimmunity is also thought to play a role. Diagnosis usually relies on the demonstration of a specific subset of circulating autoantibodies in patient serum, although in a small subset of patients, these autoantibodies might be absent. Due to its rarity, there are no set of guidelines for treatment. The general approach includes a variety of immunosuppressive agents and treatment of the underlying neoplasia. Despite treatment, paraneoplastic pemphigus ...
TY - JOUR. T1 - Unusual presentation of pemphigus vulgaris. AU - Bardazzi, F.. AU - Guerra, L.. AU - Peluso, A. M.. AU - Piraccini, B. M.. AU - Morelli, R.. PY - 1994. Y1 - 1994. N2 - We report a case of a 50-year-old man who presented with a conjunctival erosion and lesions resembling acute paronychia of several fingers. The Tzanck preparation from the conjunctiva showed the presence of acantholytic cells. Skin biopsy showed suprabasal cleft formation with acantholysis. Direct immunofluorescence revealed intercellular epidermal staining with IgG and C3. Diagnosis of pemphigus vulgaris was therefore confirmed. Nail abnormalities that can be observed in pemphigus vulgaris are discussed.. AB - We report a case of a 50-year-old man who presented with a conjunctival erosion and lesions resembling acute paronychia of several fingers. The Tzanck preparation from the conjunctiva showed the presence of acantholytic cells. Skin biopsy showed suprabasal cleft formation with acantholysis. Direct ...
Paraneoplastic pemphigus is an autoimmune acantholytic mucocutaneous disorder that rarely accompanies an overt or occult neoplasm. Typical features include painful mucosal erosive lesions and pruritic papulosquamous eruptions that often progress to blisters. The antibody is a unique immunoglobulin G that recognises epidermal proteins. We discuss the case of a 23-year-old woman who presented with anaplastic large cell lymphoma complicated by paraneoplastic pemphigus, rapidly resulting in fatal multiorgan failure.. A 23-year-old woman presented with 1 week of fever and a disseminated erythrodermic rash with desquamation of the hands and feet, and erosions in the mouth. A local doctor started treatment with ciprofloxacin and prednisolone ...
Paraneoplastic pemphigus. Close-up of paraneoplastic pemphigus (PNP) lesions on the shoulder and chest of a 60-year-old male patient with non-Hodgkins lymphoma, a form of blood cancer. PNP is an autoimmune disease resulting from an underlying tumour. It is thought that antigens associated with the tumour trigger an immune response that results in blistering of the skin near the tumour. Non-Hodgkins lymphoma is one of the two malignant tumours most commonly associated with PNP. Prognosis is poor. - Stock Image C029/2399
Background: Captopril is an angiotensin-converting enzyme inhibitor with sulphydryl groups in its chemical structure. It is commonly used as an antihypertensive drug. The occurrence of pemphigus vulgaris has repeatedly been reported in patients receiving captopril. The capacity of captopril and pemphigus serum to induce acantholysis, in vivo or in vitro, has been demonstrated experimentally. Objectives: To show that captopril and pemphigus serum, acting by a biochemical and immunological mechanism, respectively, trigger apoptosis. Methods: Human keratinocyte cells were treated with 15 mmol L)1 captopril or with pemphigus serum. DNA was extracted and the terminal deoxynucleotidyl transferase-mediated deoxyuridine triphosphate nick end labelling method was used to detect apoptosis. Results: DNA fragmentation occurred after 72 h of treatment. Increased expression of p53, c-myc and inducible nitric oxide (NO) synthase (iNOS) mRNA were observed by polymerase chain reaction (PCR) in the treated cells ...
Benign familial pemphigus, Hailey-Hailey disease, Benign familial chronic pemphigus, BFP, Familial benign chronic pemphigus, MIM 169600, Familial benign pemphigus. Authoritative facts from DermNet New Zealand.
Paraneoplastic pemphigus is an autoimmune disorder stemming from an underlying tumor. It is hypothesized that antigens associated with the tumor trigger an immune response resulting in blistering of the skin and mucous membranes. While patients with malignant and benign tumors are both at risk, malignancy is associated with high mortality rates (near 90%). Current treatment focuses on general wound healing and administering corticosteroids, which has not demonstrated a high success rate. Recent research developments aim to treat the underlying tumor in order to alleviate the symptoms of PNP. While the presence of lesions is the denominator among patients with PNP, the characteristics of the lesions differ. The five clinical presentations of lesions associated with PNP include: Pemphigus-like: Flaccid blister (discrete), crusts over the raw exuding skin lesions Pemphigoid-like: Tense blister(s) on brick red erythema Erythema multiforme-like: Severe polymorphic skin and/or mucous membrane ...
Castlemans disease is a rare autoimmune disorder with varied clinical presentations. Castlemans commonly involves mediastinum and hence it is thoracic in most of the reported cases. Paraneoplastic pemphigus (PNP) and myasthenia gravis can be associated with multicentricCastlemans disease. Its association with HIV, Kaposi sarcoma, and lymphoma is also well known. We report a rare combination of unicentric, extrathoracicCastlemans disease with PNP and myasthenia gravis.. ...
Pemphigus vulgaris is an autoimmine disorder, where the bodys immune system attacks some of the proteins in the skin. Pemphigus usually occurs in middle-aged or older people. This picture shows a close-up
Researchers at the University of North Carolina at Chapel Hill may have found a safer, more effective way to treat the life-threatening autoimmune skin disease pemphigus vulgaris without turning off the immune system.
Familial benign pemphigus pathology, Hailey-Hailey disease pathology, Familial benign chronic pemphigus pathology, MIM 169600 pathology. Authoritative facts from DermNet New Zealand.
BACKGROUND: We have shown previously that human desmocollin (Dsc) 1 is recognized by IgA autoantibodies of subcorneal pustular dermatosis (SPD) type IgA pemphigus. However, the presence of IgG anti-Dsc autoantibodies is still controversial, and antibodies to Dsc2 and Dsc3 have not been clearly identified.. OBJECTIVES: To investigate this by producing recombinant proteins consisting of the entire extracellular domains of human Dsc1, 2 and 3 in baculovirus, and to use them to establish an enzyme-linked immunosorbent assay (ELISA).. METHODS: By this ELISA, we examined in total 165 cases of various types of autoimmune bullous diseases, as well as 23 normal controls.. RESULTS: None of 45 sera of classical pemphigus showed either IgG or IgA antibodies to any Dsc. In contrast, one atypical pemphigus serum showed both IgG and IgA antibodies to Dsc1, which were adsorbed by incubation with Dsc1 baculoprotein. Furthermore, this ELISA detected both IgA and IgG anti-Dsc3 antibodies in one atypical case, and ...
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Hailey-Hailey disease, also known as benign chronic pemphigus, is a rare autosomal dominant cutaneous disorder that usually becomes manifest in the third or fourth decade of life with erythema, vesicles, and erosions involving the body folds, particularly the groin and axillary regions. Other sites of the body, such as the neck, perianal, and submammary regions, may likewise be affected (summary by Poblete-Gutierrez et al., 2004). This disorder was first described by the dermatologist brothers Hailey and Hailey (1939).
ACANTHOLYSIS: Review the definition, meaning, pronunciation, explanation, synonyms, and antonyms of the term ACANTHOLYSIS in the Online Dictionary. What is a 12 letter word that starts with A?
Isolation of monoclonal autoantibodies from PV patients. Peripheral blood samples were obtained from 2 patients (PVA and PVB) suffering from active mucocutaneous PV. The patients showed typical clinical, histological, and immunopathological features and had high-titer anti-DSG circulating autoantibodies (PVA: DSG3, 308 U/ml, DSG1, 110 U/ml; PVB: DSG3, 191 U/ml, DSG1, 170 U/ml), as assessed by ELISA kits based on ectodomain of DSG1 and DSG3 (MBL). IgG+ memory B cells were isolated from cryopreserved PBMC using CD22 microbeads (Miltenyi Biotec) followed by depletion of cells carrying IgM, IgD, and IgA by cell sorting. Multiple replicate microcultures of 10-30 IgG+ memory B cells/well (for a total of 2 to 8 × 104 purified cells) were infected with EBV and CpG as previously described (15). Culture supernatants were tested for binding to DSG3-coated ELISA plates and for binding to the keratinocyte cell line (HaCaT) monolayers by IF assay using an automated fluorescence microscope (Pathway 855; BD). ...
Founded in 2010, this research institute was established as the Kurume University Institute of Cutaneous Cell Biology. This institute was granted the Grants-in-Aid for Scientific Research and Strategic Research Basis Formation Supporting Project in Private Universities by the Ministry of Education, Culture, Sports, Science and Technology of Japan, and Health and Labour Sciences Research Grants and the grants for Research on Measures for Intractable Diseases by the Ministry of Health, Labour and Welfare of Japan in April 2009.. This institute is widely open for collaborative researchers in and outside of Kurume University, in either domestic institute or institute abroad. This institute performs many translational researches between various skin diseases and basic researches of the skin. There are three main subjects for this institute, i.e., (1) keratinocyte cell biology (mainly, pathogenicity, diagnosis and treatment of various autoimmune bullous skin diseases, (2) genetic studies of various ...
Head and neck pruritus. These are referred to nowadays as reaction patterns, because two cats may present with an identical clinical picture, but it will be identified to have a different underlying cause. In this case, the skin is reacting in an often relatively predictable fashion or pattern to the underlying stimulus. This predictable reaction can even be similar histopathologically! Miliary Dermatitis. Miliary dermatitis is characterized clinically by numerous, small, localized or generalized papules and crusted papules. It is the most common feline problem in small animal dermatology.. The list of differential diagnoses for miliary dermatitis is lengthy. Allergies such as fleabite hypersensitivity, atopy or food intolerance, ectoparasites such as Otodectes cynotis, Notoedres cati, or Cheyletiella blakei, infections with dermatophytes or bacteria, neoplastic diseases such as mast cell tumors, immune-mediated diseases such as pemphigus foliaceus and nutritional shortcomings such as ...
Anhalt GJ, Kim SC, Stanley JR, Korman NJ, Jabs DA, Kory M, Izumi H, Ratrie H III, Mutasim D, Ariss-Abdo L, and Labib RS: A novel autoimmune mucocutaneous syndrome, associated with underlying neoplasia: Paraneoplastic Pemphigus. N Engl J Med 323:1729-1735, 1990 ...
A 27 year-old man presents with numerous erosions of his mucous membranes. Examination of his ... d. Pemphigus vulgaris e. Bullous pemphigoid
I think it would be good idea to use oral prednisolone up to 20mg tid for 1-2 weeks and once the disease is under control, slowly taper off the prednisolone till the lowest dose to control the disease. However if the disease relapses on cutting down oral prednisolone, I would consider adding either low dose weekly methotrexate or azathioprine. There are other drugs like cyclophosphamide and cell cept but need to consider their toxicity and cost involved. You would need to look at the total number of lesions and assess whether they are severe enough to interfere with his daily activities ...
Skin tags are harmless growths on the skin that can vary in number. They are usually the same color as your skin or slightly darker. These tiny pieces of tissue are composed of blood vessels and a type of protein fiber called collagen. They project from the surrounding skin on a thin or thick stalk. While most skin tags are small, pinhead-sized bumps, they may become as large as a grape Skin tags can develop on any part of the body, but they most commonly grow on areas of high friction or areas that are commonly rubbed, such as, neck, breasts, groin, stomach, eyelids, underarms. Males and females are equally prone to getting skin tags. However, people may be more likely to develop skin tags if they are obese, are pregnant, or have diabetes Causes--Researchers dont know exactly what causes skin tags to grow. It is believed that friction may lead to the development of skin tags. The growths commonly occur in areas where skin constantly rubs against clothing or other skin, such as near yo ...
Dr Tan Hiok Hee is a Dermatologist with expertise in various aspects of medical and cosmetic dermatology. He completed his MBBS from the National University of Singapore. He was formerly a Senior Consultant and Assistant Director at the National Skin Centre, Singapore. He also helmed the Department of Sexually Transmitted Infections (STI) Control and was also Chief of the Cutaneous Infections unit at NSC, and the Consultant in charge of the Acne clinic.. During his time as a registrar undergoing specialist training, Dr Tan Hiok Hee was the recipient of two National Medical Research Council grants, for which he conducted research on the use of molecular techniques in STI diagnosis and antibiotic resistance amongst acne patients.. He also received the Best Research Paper award from the Dermatological Society of Singapore for 3 consecutive years. In 2001, Dr Tan Hiok Hee completed a fellowship with St Johns Institute of Dermatology and the Department of Genitourinary Medicine at Guys & St ...
Pemphigus vulgaris is exhibited by blistering and sores on the skin and mucus membranes. The immune system produces antibodies against specific proteins in the skin and mucus membranes, and the antibodies break the bonds between skin cells. This leads to the formation of a blister. About half of sufferers first develop painful blisters and sores in the mouth, followed by blisters on the skin that may come and go. It is 40 times more common among Jews than in the general population of the world ...
A tongue that is in good shape is taken as a prime indicator of your overall body being in the pink of health, and vice versa. So, conditions like tongue blisters can very well be symptomatic of some other grave health scare that might merit medical attention. This makes it all the more necessary not to take them lightly, and see a doctor immediately to ascertain the cause and get it treated accordingly.. The general rule of thumb recommended by medical experts is that if the blisters persist for longer than 2 weeks despite the prescribed primary treatment and care, the discomfort becomes unmanageable such that it keeps you from going about your daily business, or the condition tends to worsen progressively and is accompanied by fever. It is best to seek the help of your dentist to rule out the possibility of an oral infection or conditions that are rarer, such as pemphigus vulgaris or oral cancer.. In general, tongue blisters last for about a week and disappear on their own. However, you can ...
Autosomal dominant inherited skin disease with variable gene penetration characterized by recurrent eruptions of vesicles and bullae mainly in intertriginous areas. The neck, axillae and the inguinal or perianal region are typically involved. Lesions show itchy patches with scaling crusts and fissured surfaces. They extend peripherically and the centre may heal or show soft, flat, moist vegetations. The course is chronically recurrent with remissions. Complications due to secondary infection may occur. A number of external stimuli have been shown to produce lesions including friction, freezing and ultraviolet radiation. The disorder is unrelated to pemphigus vulgaris.. ...
Human class II pMHC tetramers containing autoantigenic specificities have been used to study T cell responses in a variety of diseases, including type 1 diabetes (T1D), celiac disease, pemphigus vulgaris, rheumatoid arthritis, multiple sclerosis, and uveitis (37-47). These studies have highlighted a fundamental point: the avidity of the TCR interaction with its pMHC target is influenced by selection against high-affinity self-Ag recognition. Unlike tetramer analyses in the infectious pathogen and allergen studies cited above, in which most of the responding T cells have a high-avidity binding to appropriate pMHC tetramers, tetramer binding to peripheral T cells in the context of autoimmunity displays a much wider spectrum of relative strength of interaction. In addition, the frequency of autoreactive CD4 T cells specific for a particular pMHC-self-Ag complex is quite low in peripheral blood, often less than five per million lymphocytes, requiring large sample volumes and careful handling for ...
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Faith is a 7-year-old Jack Russell Terrier with canine autoimmune disease, pemphigus. She has no hair on her head, ankles or tail (except for a few wispy, white ones on the bottom ...
Faith is a 7-year-old Jack Russell Terrier with canine autoimmune disease, pemphigus. She has no hair on her head, ankles or tail (except for a few wispy, white ones on the bottom ...
Faith is a 7-year-old Jack Russell Terrier with canine autoimmune disease, pemphigus. She has no hair on her head, ankles or tail (except for a few wispy, white ones on the bottom ...
I have just been diagnosed with Pemphigus. I dont know what to expect. I see my doctor on Friday for the first time. I am always tired. Is this a symptom of...
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Patch testing is sometimes indicated. It should only be performed by a specialist practitioner, as reactions may be severe.. ...