ArticleAnais brasileiros de dermatologia
Epidermolysis bullosa acquisita.
Article in Anais brasileiros de dermatologia. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers, 1 of them a synthesis that pooled it.
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Who cites it
14 citing papers in PubMed, 1 synthesis or guideline pooled it, 35 citations in OpenAlex.
- Diagnostic Agreement in Subepidermal Blistering Diseases: Is ELISA Test Reliable as Direct Immunofluorescence? A Systematic Review, Meta-Analysis, and Trial Sequential Analysis.Journal of oral pathology & medicine : official publication of the International Association of Oral Pathologists and the American Academy of Oral Pathology · 2026Pooled it
- Epidermolysis bullosa acquisita improved with use of dupilumab.JAAD case reports · 2026Article
- Recombinant humanized type VII collagen for skin repair and regeneration: prospects for reconstructing the dermal-epidermal junction.Frontiers in medicine · 2026Review
- Oral Manifestations of Autoimmune Diseases.Advances in experimental medicine and biology · 2026Review
- Colchicine in Contemporary Pharmacotherapy: Mechanistic Insights and Clinical Horizons.Journal of clinical medicine · 2025Review
- Article
- From Multiple Sclerosis to Organ-Specific Autoimmune Disorders: Insights into the Molecular and Clinical Implications of Comorbidity.Molecular neurobiology · 2025Review
- Immunological control of skin development: from homeostasis to developmental pathologies.Frontiers in immunology · 2025Review
- A Novel COL7A1 Mutation in a Patient With Dystrophic Epidermolysis Bullosa. Successful Treatment With Upadacitinib.Clinical, cosmetic and investigational dermatology · 2025Article
- IgM-mediated epidermolysis bullosa acquisita.JAAD case reports · 2024Article
- Oleogel-S10 in Dystrophic Epidermolysis Bullosa: A Case Series Evaluating the Impact on Wound Burden Over Two Years.Advances in therapy · 2024Article
- Anti-Idiotypic mRNA Vaccine to Treat Autoimmune Disorders.Vaccines · 2023Review
- Intravenous Immunoglobulin for Autoimmune Bullous Diseases: A Case Series from a Central European Referral Center.Medicina (Kaunas, Lithuania) · 2023Article
- Retrospective analysis of autoimmune bullous diseases in Middle Franconia.Frontiers in immunology · 2023Article
Corrections and comments
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Authors and funding
4 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Epidermolysis bullosa acquisita is a rare autoimmune disease, characterized by the synthesis of anti-collagen VII autoantibodies, the main component of hemidesmosome anchoring fibrils. The antigen-antibody binding elicits a complex inflammatory response, which culminates in the loss of dermo-epidermal adhesion of the skin and/or mucous membranes. Skin fragility with bullae, erosions, and milia in areas of trauma characterizes the mechanobullous form of the disease. In the inflammatory form of epidermolysis bullosa acquisita, urticarial inflammatory plaques with tense bullae, similar to bullous pemphigoid, or mucosal lesions can determine permanent scars and loss of functionality in the ocular, oral, esophageal, and urogenital regions. Due to the similarity of the clinical findings of epidermolysis bullosa acquisita with other diseases of the pemphigoid group and with porphyria cutanea tarda, the diagnosis is currently confirmed mainly based on the clinical correlation with histopathological findings (pauci-inflammatory subepidermal cleavage or with a neutrophilic infiltrate) and the demonstration of the presence of anti-collagen VII IgG in situ by direct immunofluorescence, or circulating anti-collagen VII IgG through indirect immunofluorescence and/or ELISA. There is no specific therapy for epidermolysis bullosa acquisita and the response to treatment is variable, usually with complete remission in children and a worse prognosis in adults with mucosal involvement. Systemic corticosteroids and immunomodulators (colchicine and dapsone) are alternatives for the treatment of mild forms of the disease, while severe forms require the use of corticosteroid therapy associated with immunosuppressants, intravenous immunoglobulin, and rituximab.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.