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 Advances in understanding and managing bullous pemphigoid

F1000Research ◽  
2015 ◽  
Vol 4 ◽  
pp. 1313 ◽  
Author(s):  
Cathy Y. Zhao ◽  
Dedee F. Murrell
Keyword(s):  
Elderly Patients ◽  
Bullous Pemphigoid ◽  
Outcome Measures ◽  
Diagnostic Tests ◽  
Therapeutic Options ◽  
Autoimmune Blistering Disease ◽  
Recent Advances ◽  
New Knowledge ◽  
The World ◽  
Blistering Disease

Bullous pemphigoid (BP) is the commonest subtype of autoimmune blistering disease in most countries of the world. It occurs most frequently in elderly patients and is characterised clinically by large, tense blisters in the skin preceded by urticarial plaques and pruritus. Immunopathologically, it is characterised by autoantibodies directed against the 180 kD antigen (BP180) and the 230 kD antigen (BP230). New knowledge regarding BP is being continually uncovered. This article reviews the recent advances in BP, including newer diagnostic tests, standardised outcome measures and emerging therapeutic options, as well as the evidence supporting their use.

scholarly journals The Role of Intereukin-31 in Pathogenesis of Itch and Its Intensity in a Course of Bullous Pemphigoid and Dermatitis Herpetiformis

2017 ◽  
Vol 2017 ◽  
pp. 1-8 ◽  
Author(s):  
Lilianna Kulczycka-Siennicka ◽  
Anna Cynkier ◽  
Elżbieta Waszczykowska ◽  
Anna Woźniacka ◽  
Agnieszka Żebrowska
Keyword(s):  
Signaling Pathway ◽  
Bullous Pemphigoid ◽  
Dermatitis Herpetiformis ◽  
Subjective Symptoms ◽  
Autoimmune Blistering Disease ◽  
Stat Signaling ◽  
Blistering Disease ◽  
Autoimmune Blistering Diseases

Itch which is one of the major, subjective symptoms in a course of bullous pemphigoid and dermatitis herpetiformis makes those two diseases totally different than other autoimmune blistering diseases. Its pathogenesis is still not fully known. The aim of this research was to assess the role of IL-31 in development of itch as well as to measure its intensity. Obtained results, as well as literature data, show that lower concentration of IL-31 in patients’ serum may be correlated with its role in JAK/STAT signaling pathway which is involved in development of autoimmune blistering disease. Intensity of itch is surprisingly huge problem for the patients and the obtained results are comparable with results presented by atopic patients.

scholarly journals Juvenile Bullous Pemphigoid – a Case Report

2013 ◽  
Vol 5 (1) ◽  
pp. 31-36
Author(s):  
Kristina Kostić ◽  
Lidija Kandolf Sekulović ◽  
Radoš D. Zečević
Keyword(s):  
Bullous Pemphigoid ◽  
White Cell Count ◽  
Direct Immunofluorescence ◽  
Elderly Persons ◽  
Dermoepidermal Junction ◽  
Autoimmune Blistering Disease ◽  
Systemic Corticosteroids ◽  
Laboratory Test Results ◽  
Intense Pruritus ◽  
Blistering Disease

Abstract Bullous pemphigoid is an autoimmune blistering disease that predominantly affects elderly persons and rarely children. We present a 12-year-old girl with sudden appearance of tense blisters on an erythematous base on the trunk, neck, hands and legs with intense pruritus. Standard laboratory test results were within the normal range except for blood eosinophilia of 12% of the total white cell count. Skin biopsy specimens showed evolving subepidermal blisters with perivascular lymphohistiocytic, eosinophil and neutrophil infiltrations in the papillary dermis. Direct immunofluorescence of perilesional skin showed linear, continuous deposits of IgG and C3 along the dermoepidermal junction. Indirect immunofluorescence showed circulating anti-basement membrane zone IgG autoantibodies at a titer of 1:80. We started treatment with systemic corticosteroids, methylprednisolone 0,5 mg/kg per day and 500 mg erythromycin 4 times a day during 10 days. After 3 days 50 mg dapsone (DDS, 4,4-diaminodiphenylsulphone) per day was added. After a few days, there were no new changes on the skin and pruritus disappeared completely.

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.

Role of complement cascade in experimental autoimmune blistering disease bullous pemphigoid

2007 ◽  
Vol 44 (1-3) ◽  
pp. 158
Author(s):  
Kelly E. Carter ◽  
Pamela A. Rose ◽  
Minglang Zhao ◽  
Rick A. Wetsel ◽  
Luis A. Diaz ◽  
...  
Keyword(s):  
Bullous Pemphigoid ◽  
Complement Cascade ◽  
Autoimmune Blistering Disease ◽  
Blistering Disease ◽  
Experimental Autoimmune

scholarly journals Bullous pemphigoid autoantibodies

2021 ◽  
Vol 5 (4) ◽  
pp. 259-263
Author(s):  
Florentina-Silvia Delli ◽  
◽  
Elena Sotiriou ◽  
Efstratios Vakirlis ◽  
Demetrios Ioannides
Keyword(s):  
Bullous Pemphigoid ◽  
Dermatitis Herpetiformis ◽  
Direct Immunofluorescence ◽  
Risk Of Death ◽  
Class Switch ◽  
Autoimmune Blistering Disease ◽  
Systemic Corticosteroids ◽  
First Choice ◽  
Blistering Disease ◽  
Blistering Skin Disorders

<abstract> <p>Autoimmune blistering skin disorders are rare. According to direct immunofluorescence studies, three categories are described: pemphigus group, pemphigoid group and dermatitis herpetiformis. Among these diseases, bullous pemphigoid is the most common. Patients with typical bullous pemphigoid disease are usually elderly and have many comorbidities. Considering that topical and systemic corticosteroids are the first choice therapy, these patients also have increased morbidity and risk of death. The main characteristic of bullous pemphigoid as an acquired autoimmune blistering disease is the formation of autoantibodies against hemidesmosomal antigens BP180 and BP230. Although IgG autoantibodies predominate within the plasma and skin of BP patients, some features of the disease cannot be explained solely by IgG-mediated mechanisms. Epitope spreading phenomena, immunoglobulin class switch and the relevance of IgM and IgE autoantibodies are discussed in this article.</p> </abstract>

scholarly journals Increased risk of venous thromboembolism in patients with bullous pemphigoid

2016 ◽  
Vol 115 (01) ◽  
pp. 193-199 ◽  
Author(s):  
Paolo Bucciarelli ◽  
Ylenia Balice ◽  
Giuseppe Cianchini ◽  
Pietro Quaglino ◽  
Piergiacomo Calzavara Pinton ◽  
...  
Keyword(s):  
Risk Factors ◽  
Venous Thromboembolism ◽  
Bullous Pemphigoid ◽  
Acute Phase ◽  
Hazard Ratio ◽  
Autoimmune Blistering Disease ◽  
Cox Proportional Hazard ◽  
Increased Risk ◽  
Blistering Disease

SummaryActivation of blood coagulation has been demonstrated in bullous pemphigoid (BP), a rare autoimmune blistering disease, potentially leading to a prothrombotic state. In order to evaluate the incidence of venous thromboembolism (VTE) in BP, a cohort study was carried out on 432 BP patients (59 % females; median age 76 years, interquartile range [IQR]: 68–82). At diagnosis, autoimmune bullous skin disorder intensity score (ABSIS) was calculated. VTE incidence was standardised with rates of the general population. Multivariable Cox proportional hazard model was used to estimate the hazard ratio of VTE according to ABSIS and concomitant risk factors. During a median follow-up of 4.2 years, 31 objectively-diagnosed VTE events were recorded. The incidence rate of VTE (per 1000 patient-years) was 17.2 overall (95 % confidence interval [CI]: 11.1–23.2), 56.7 (95 %CI: 33.0–80.4) during acute phase (22 VTE) and 6.3 (95 %CI: 2.8–11.3) during remission (9 VTE). The standardised incidence ratio was 4.06 (95 %CI: 2.73–5.65), higher during the acute phase (14.86, 95 %CI: 9.20–21.88) than during remission (1.48, 0.66–2.63). The adjusted hazard ratio of VTE was 2.74 (95 %CI: 1.07–7.04) for ABSIS > 48 vs ABSIS < 28, and 2.56 (95 %CI: 1.00–6.70) in patients with [uni2265] 2 concomitant risk factors. In conclusion, BP patients have a 15-fold increased VTE risk during acute phase, proportional to disease severity and heightened by concomitant risk factors.

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