Clinical and Serological Characterization of Orf-Induced Immunobullous Disease

Kaan Yilmaz, Stephanie Goletz, Henri H. Pas, Renate R. Van Den Bos, Andrew Blauvelt, Wain L. White, Jean David Bouaziz, Elina Zuelgaray, Maryam Daneshpazhooh, Kim B. Yancey, Matthias Goebeler, Enno Schmidt*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

6 Citations (Scopus)


Importance: Ecthyma contagiosum, or orf, is a viral zoonotic infection caused by Poxviridae. Although human orf infection is considered to follow a self-limited course, various immunological reactions may be triggered, including immunobullous diseases. In the majority of the latter cases, the antigenic target remained enigmatic. Objective: To characterize the predominant autoantigen in orf-induced immunobullous disease and further describe this clinical entity. Design, Setting, and Participants: This multicenter case series sought to provide detailed clinical, histopathological and immunological characteristics of a patient with orf-induced pemphigoid. Based on this index patient, serological analyses were conducted of 4 additional patients with previously reported orf-induced immunobullous disease. Immunoblotting with extracellular matrix and a recently established indirect immunofluorescence assay for detection of serum anti-laminin 332 IgG were performed. Exposures: The disease course and clinical characteristics of orf-induced immunobullous disease were observed. Main Outcomes and Measures: Orf-induced immunobullous disease is primarily characterized by anti-laminin 332 autoantibodies, predominant skin involvement, and a self-limiting course. The study provides further details on epidemiological, clinical, immunopathological, diagnostic, and therapeutic aspects of orf-induced immunobullous disease. Results: In all 5 patients, IgG1 and/or IgG3 autoantibodies against laminin 332 were identified. The α3, β3, and γ2 chains were recognized in 2, 4, and 1 patient(s), respectively. Conclusions and Relevance: In this case series, laminin 332, a well-known target antigen in mucous membrane pemphigoid, was a major autoantigen in orf-induced immunobullous disease, even though predominant mucosal lesions were lacking in this autoimmune blistering disease. Orf-induced anti-laminin 332 pemphigoid is proposed as distinct clinical entity..

Original languageEnglish
Pages (from-to)670-674
Number of pages5
JournalJAMA Dermatology
Issue number6
Early online date30 Mar 2022
Publication statusPublished - Jun 2022

Bibliographical note

Funding Information:
supported by structural funding from the Deutsche Forschungsgemeinschaft (DFG) through CRU 303 Pemphigoid Diseases and the Excellence Cluster 2167/1 Precision Medicine in Chronic Inflammation (to Dr Schmidt).

Publisher Copyright:
© 2022 American Medical Association. All rights reserved.


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