ArticleCureus2025
Complete Resolution of Atypical Paraneoplastic Pemphigus Following Treatment With Dupilumab.
Article in Cureus, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
1 citing paper in PubMed.
- Clinical improvement of pemphigus-like mucocutaneous disease in a patient with CLL following initiation of zanubrutinib: a case report.Frontiers in immunology · 2026Article
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Authors and funding
4 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Paraneoplastic pemphigus (PNP) is a rare autoimmune mucocutaneous blistering condition associated with underlying malignancies. While severe stomatitis is usually a hallmark feature of PNP, it may present with the absence of oral manifestations. Proposed diagnostic criteria include the presence of mucosal lesions with or without cutaneous involvement, concomitant internal neoplasm, anti-plakin autoantibodies, histopathology with acantholysis and/or lichenoid interface changes, and direct immunofluorescence (DIF) with intercellular and/or basement membrane deposition of immunoglobulin G (IgG) or complement component-3 (C3). Management of PNP traditionally involves immunosuppressants; however, prognosis remains poor. This report presents a 77-year-old male patient with an ongoing, intensely painful, and pruritic rash with diffuse scaling, erythema, crust, bullae, and erosions covering 90% body surface area that notably lacked mucosal involvement. Biopsies revealed acantholysis with mixed infiltrate lichenoid interface dermatitis and DIF with 3+ intercellular and basement membrane deposition of C3 and IgG. Additional workup revealed elevated desmoglein-1 and bullous pemphigoid antigen-1 (BPAG1/BP230) autoantibodies and intramucosal gastrointestinal adenocarcinoma, satisfying the diagnostic criteria for PNP. Due to his history of type 2 diabetes mellitus, chronic non-healing ulcers, and untreated malignancy, he was a poor candidate for first-line immunosuppressants. Additionally, the patient experienced significant pruritus resistant to topical corticosteroids and oral antihistamines. For these reasons, dupilumab was elected to control the patient's pruritus. After two months, there was significant improvement in skin lesions, and after four months, there were complete resolution of pruritus and 100% clearance of skin lesions. This report highlights a unique and previously unreported case of complete resolution of atypical PNP following empiric treatment with dupilumab, a monoclonal antibody against interleukin 4/13 receptor-α.
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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.