scholarly journals Anti-p200 pemphigoid: A review

2021 ◽  
Vol 0 ◽  
pp. 1-6
Author(s):  
Vattiyamveetil Sajeela Rasheed
Keyword(s):  
Clinical Features ◽  
Autoimmune Blistering Disease ◽  
Mucosal Involvement ◽  
Blistering Disease ◽  
Circulating Autoantibodies

Anti-p200 pemphigoid, initially described in 1996, is a subepidermal autoimmune blistering disease. It is manifested as tense blisters, mostly in an acral distribution, and is accompanied by mucosal involvement in more than half the cases. The disease is produced by circulating autoantibodies directed against the dermal antigen of 200-kDa, the exact identity of which remains unknown. This review focuses on the clinical features, immunopathogenesis, and diagnosis of anti-p200 pemphigoid.

scholarly journals Immunization with desmoglein 3 induces non-pathogenic autoantibodies in mice

PLoS ONE ◽  
2021 ◽  
Vol 16 (11) ◽  
pp. e0259586
Author(s):  
Katharina Boch ◽  
Sören Dräger ◽  
Detlef Zillikens ◽  
Christoph Hudemann ◽  
Christoph M. Hammers ◽  
...  
Keyword(s):  
Disease Model ◽  
Pemphigus Vulgaris ◽  
Conformational Epitope ◽  
Autoimmune Blistering Disease ◽  
Immunization Strategies ◽  
Blistering Disease ◽  
Native Antigen ◽  
Circulating Autoantibodies ◽  
Monkey Esophagus

Background Pemphigus vulgaris (PV) is a rare autoimmune blistering disease characterized by the development of autoantibodies targeting desmoglein (Dsg) 3, but also against Dsg1 in mucocutaneous disease. Given that existing PV animal models only recapitulate aspects of the disease, we aimed to establish a more comprehensive disease model based on the immunization of mice with PV autoantigen(s). Methods The following immunization strategies were tested: (i) C57Bl/6J, B6.SJL-H2s C3c/1CyJ, DBA2/J, or SJL/J mice were immunized with recombinant murine Dsg3 (mDsg3), (ii) DBA2/J and SJL/J mice were immunized with mDsg3 and additionally injected a single non-blister inducing dose of exfoliative toxin A (ETA), and (iii) DBA2/J and SJL/J mice were immunized with human Dsg (hDsg) 1 and 3. Results Despite the induction of autoantibodies in each immunization protocol, the mice did not develop a clinical phenotype. Tissue-bound autoantibodies were not detected in the skin or mucosa. Circulating autoantibodies did not bind to the native antigen in indirect immunofluorescence microscopy using monkey esophagus as a substrate. Conclusion Immunization with PV autoantigens induced non-pathogenic Dsg1/3 antibodies, but did not cause skin/mucous membrane disease in mice. These findings, confirmed by failure of binding of the induced autoantibodies to their target in the skin, suggest that the autoantibodies which were formed were unable to bind to the conformational epitope present in vivo.

scholarly journals Esophageal Nikolsky’s Sign: A Rare Finding in a Patient with Bullous Pemphigoid

2019 ◽  
Vol 13 (3) ◽  
pp. 445-449 ◽  
Author(s):  
Sara Ghoneim ◽  
Aun Shah ◽  
Amy Calderon
Keyword(s):  
Elderly Patients ◽  
Bullous Pemphigoid ◽  
Middle Age ◽  
Epigastric Pain ◽  
Mucosal Injury ◽  
Rare Finding ◽  
Esophageal Involvement ◽  
Autoimmune Blistering Disease ◽  
Mucosal Involvement ◽  
Blistering Disease

Bullous pemphigoid (BP) is a chronic relapsing autoimmune blistering disease that typically affects middle-age and elderly patients. It can manifest with varying degrees of mucosal involvement in addition to characteristic skin findings. However, esophageal involvement is very rare. We report a case of a 57-year-old female with BP who presented with epigastric pain and melena. She underwent an esophagogastroduodenoscopy which induced bullae seen only upon withdrawal of the endoscope. This finding is analogous to the dermatological finding of Nikolsky’s sign. Gentle insertion and withdrawal of the endoscope is recommended to reduce the risk of bullae formation and mucosal injury.

scholarly journals An immunologic intrigue: psoriasis vulgaris developing on pemphigus foliaceous

2021 ◽  
Vol 7 (4) ◽  
pp. 609
Author(s):  
Deepali C. Tarate ◽  
Rachita Misri ◽  
Vinod K. Khuranna
Keyword(s):  
Rare Case ◽  
Psoriasis Vulgaris ◽  
First Report ◽  
Autoimmune Blistering Disease ◽  
Autoimmune Bullous Diseases ◽  
Mucosal Involvement ◽  
The Face ◽  
Blistering Disease ◽  
Bullous Diseases

<p class="abstract">Pemphigus foliaceous is an autoimmune blistering disease characterized by superficial flaccid blisters, erosions, scales and crusts on the seborrheic areas of the face and trunk. Mucosal involvement is seldom seen. The first report by Bloom in 1929, stated the coexistence of pemphigus foliaceous and psoriasis vulgaris. Since then, very few cases of psoriasis coexistent with autoimmune bullous diseases have been reported in which psoriasis had preceded or simultaneously presented with pemphigus foliaceous. We reported a rare case where psoriasis vulgaris developed in case of pemphigus foliaceous after 20 years.</p>

Autoimmune Blistering Disease with Concurrent IgG Antibodies to Desmoglein 1 and BP180 : Two Cases

2013 ◽  
Vol 75 (5) ◽  
pp. 415-418
Author(s):  
Miki ICHIKAWA ◽  
Maya TANAKA ◽  
Kazunori URABE ◽  
Masutaka FURUE
Keyword(s):  
Igg Antibodies ◽  
Autoimmune Blistering Disease ◽  
Blistering Disease

Sweet Syndrome and Pemphigus Vulgaris

2012 ◽  
Vol 16 (2) ◽  
pp. 128-130 ◽  
Author(s):  
Ashley O'toole ◽  
Maureen O'malley
Keyword(s):  
Clinical Presentation ◽  
Pemphigus Vulgaris ◽  
Neutrophilic Dermatosis ◽  
Rare Presentation ◽  
Sweet Syndrome ◽  
Autoimmune Blistering Disease ◽  
Mucous Membranes ◽  
Blistering Disease ◽  
Erythematous Plaques ◽  
New Onset

Background: Pemphigus vulgaris is an autoimmune blistering disease of the skin and mucous membranes. Sweet syndrome is an uncommon reactive neutrophilic dermatosis. Objective: This case report describes the rare presentation of a 58-year-old female patient with newly diagnosed pemphigus vulgaris who later presented with erythematous plaques and leukocytosis highly suggestive of Sweet syndrome. Methods: Review of the literature using the PubMed and Medline databases. Results: The clinical presentation of new-onset Sweet syndrome in a patient with pemphigus has been reported in the literature only on one other occasion. Conclusion: The observation of an association between Sweet syndrome and autoimmune diseases, such as pemphigus vulgaris, may ultimately lead to better understanding of the pathophysiology of this disease.

scholarly journals Pemphigus herpetiformis : A rare clinical variant of pemphigus

2016 ◽  
Vol 13 (1) ◽  
pp. 61-65
Author(s):  
P Shrestha ◽  
R B Tajhya ◽  
A Pokharel
Keyword(s):  
Inflammatory Infiltrate ◽  
Autoimmune Blistering Disease ◽  
Neutrophilic Dermatoses ◽  
Clinical Variant ◽  
Blistering Disease ◽  
Anti Inflammatory

Pemphigus herpetiformis is an autoimmune blistering disease. We report a case of pemphigus herpetiformis, a generalized form, manifested as pruritic grouped vesicles at extremities and trunk, in a seventy years old man. Neutrophil dominant inflammatory substrate was seen in histopathology. Direct immunofluorescene revealed IgG reactivity in net like pattern at upper epidermis, confirming pemphigus. The patient responded well to prednisolone and colchicine combination initially and maintained remission later on colchicine alone. Colchicine has anti-mitotic, anti-inflammatory and immunosuppressive mechanism of actions and proven benefits in neutrophilic dermatoses. Therefore we recommend colchicine as mono therapy or in combination with immunosuppressive, for the treatment of pemphigus herpetiformis where neutrophil is predominant inflammatory infiltrate histologically.NJDVL Vol. 13, No. 1, 2015 Page: 61-65

scholarly journals Blistering Diseases in a Tertiary Care Hospital: A 10-year Audit

2020 ◽  
Vol 18 (1) ◽  
pp. 24-27
Author(s):  
Niraj Parajuli ◽  
Laila Lama ◽  
Anupama Karki ◽  
Rushma Shrestha ◽  
Sumida Tiwari ◽  
...  
Keyword(s):  
Hospital Stay ◽  
Tertiary Care ◽  
Length Of Hospital Stay ◽  
Referral Hospital ◽  
Care Hospital ◽  
Medical Sciences ◽  
Mortality And Morbidity ◽  
Autoimmune Blistering Disease ◽  
Dermatological Disease ◽  
Blistering Disease

Introduction: Cutaneous blisters can occur in variety of conditions. They are broadly classified as immunobullous or non-immunobullous. Immunobullous blistering diseases can further classify as intraepidermal or subepidermal. Non-immunobullous blistering disease are commonly due to infections, drugs among others. Some of the blistering disease carry significant mortality and morbidity. There are only limited data on blistering diseases from Nepal, so this study will provide details on the prevalence of this rare dermatological disease from one of the tertiary care referral hospital in the country. Objective: To assess the clinical pattern and prevalence of various blistering diseases in a tertiary care referral hospital. Materials and Methods: All of the data were obtained from the admission register maintained at department of dermatology and venereology, National Academy of Medical Sciences. All the patients admitted with blistering disease from year April 2008 till March 2018 were included. The details including age, sex, type of blistering disease and length of hospital stay were recorded.  Results: A total of 710 patients were admitted in the dermatology ward over the period of 10 years.  There were a total of 193 blistering disease admitted during this period. Autoimmune blistering disease was diagnosed in 59 patients whereas 100 patients had non-immunobullous disease. The age grouped range from 4- 93 years with the mean age of 43± 19.71 years. The average number of hospital stay of all blistering diseases was 11.73±11.36 days. Conclusion: Blistering diseases are one of the most common conditions requiring admission in dermatology ward. Some of these diseases carry a significant morbidity and mortality. So, prompt diagnosis and treatment is of utmost importance.

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