Diagnosis and Management of Autoimmune Bullous Diseases

A special issue of Diagnostics (ISSN 2075-4418). This special issue belongs to the section "Pathology and Molecular Diagnostics".

Deadline for manuscript submissions: closed (31 October 2023) | Viewed by 1805

Special Issue Editors


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Guest Editor
Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA
Interests: autoimmune disease; pemphigus; psoriasis; lichen planus; bullous pemphigoid

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Guest Editor
Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran
Interests: dermatology; autoimmune bullous diseases

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Guest Editor
Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran
Interests: autoimmune bullous skin diseases; autoimmunity; genodermatoses; skin cancers

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Guest Editor
Department of Dermatology and Cutaneous Biology, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA
Interests: medical genetics; dermatology; genodermatoses; cutaneous warts

Special Issue Information

Dear Colleagues, 

Autoimmune bullous diseases (AIBDs) are a heterogeneous group of diseases characterized by cutaneous and mucosal vesicles and bullae. The diagnostically Relevant Clinical and Immunopathological Characteristics of Major AIBD include Pemphigus Vulgaris and Pemphigus Foliaceus, Bullous Pemphigoid, Mucous Membrane Pemphigoid, Paraneoplastic Pemphigus, Linear IgA Disease, and Epidermolysis Bullosa Acquisita, Dermatitis Herpetiformis and so on. Some clinical signs are typical for a specific autoimmune bullous disease, however, correct diagnosis requires the detection of tissue-bound or circulating autoantibodies. Diagnosis is based on a combination of clinical, histopathological, and immunofluorescence findings. Detection of autoantibodies against the specific antigens in the serum and penetrated autoantibodies into the lesions are gold standards for diagnosis and differentiate between the subtypes of AIBDs. However, there are some patients with different clinical and autoantibody patterns, which has made the diagnosis of such patients challenging. Current treatment strategies, including systemic corticosteroids and other immunosuppressants could significantly reduce disease morbidity and mortality. However, such treatments cause several side-effects due to off-target effects. During the last decade, more specific agents to target B cells and B cells’ responses, such as  anti-CD20 and Anti-CD19 monoclonal antibodies as well Bruton's tyrosine kinase inhibitors have received significant attention. In addition, more targeted therapies, such as engineering of cells are emerging. Still there is no consensus on the best approach to manage patients and there are some uncertainties about choosing the best treatments for patients with different clinical manifestations and disease severity. Our Special Issue will focus on the epidemiology, pathogenesis, potential triggers, clinical features, diagnosis and treatment of autoimmune bullous diseases. We encourage authors to submit their original research, including preclinical, translational studies, and clinical trials. Review articles and case reports/series may also be considered.

Dr. Soheil Tavakolpour
Prof. Dr. Maryam Daneshpazhooh
Dr. Hamidreza Mahmoudi
Dr. Hassan Vahidnezhad
Guest Editors

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Published Papers (1 paper)

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7 pages, 3587 KiB  
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Addressing Challenges in Diagnosis, Differential Diagnosis, and Treatment of Pemphigus: A Case Series
by Zulfa Fidi Pranadwista, Etis Duhita Rahayuningtyas and Irna Sufiawati
Diagnostics 2023, 13(24), 3633; https://doi.org/10.3390/diagnostics13243633 - 08 Dec 2023
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Abstract
Pemphigus is a rare autoimmune disease characterized by skin blisters and erosions, with or without mucosal involvement. The clinical presentation of pemphigus can resemble other bullous diseases, leading to challenges in diagnosis. This report aims to address the challenges in diagnosing and treating [...] Read more.
Pemphigus is a rare autoimmune disease characterized by skin blisters and erosions, with or without mucosal involvement. The clinical presentation of pemphigus can resemble other bullous diseases, leading to challenges in diagnosis. This report aims to address the challenges in diagnosing and treating oral pemphigus. Three patients, ranging in age from 26 to 55 years, complained of a sore throat and mouth canker sores. Extra-oral examination revealed dry lips in case 1, while serosanguinolenta crust on the lip that bled easily was found in case 2. Intra-oral examinations in all cases showed multiple painful, sloughing-covered, erosive lesions on the entire oral mucosa. The histopathological examination of case 1 revealed pemphigus foliaceous, whereas cases 2 and 3 showed pemphigus vulgaris. Secondary syphilis-like pemphigus was given as a differential diagnosis in case 2 due to the histopathological changes not being specific. The patients were instructed to maintain oral hygiene and treated with corticosteroid, analgesic, antifungal, and anti-inflammation mouthwash, as well as vitamins and minerals. All cases showed improvement in oral lesions within 14 days to a month. In conclusion, pemphigus may mimic other bullous diseases, making diagnosis challenging. A comprehensive clinical and laboratory assessment is necessary to provide accurate diagnosis and treatment. Full article
(This article belongs to the Special Issue Diagnosis and Management of Autoimmune Bullous Diseases)
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