Rapid response of IgA pemphigus of subcorneal pustular dermatosis type to treatment with isotretinoin

Claus Gruss, Detlef Zillikens, Takashi Hashimoto, Masayuki Amagai, Maximilian Kroiss, Thomas Vogt, Michael Landthaler, Wilhelm Stolz

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34 Citations (Scopus)

Abstract

Diagnosing IgA pemphigus and distinguishing between its 2 subtypes, intraepidermal neutrophilic IgA dermatosis type and subcorneal pustular dermatosis type, is important because treatment of IgA pemphigus has to be different from treatment of other blistering autoimmune dermatoses. We present a patient with subcorneal pustular dermatosis type of IgA pemphigus who rapidly responded to systemic treatment with isotretinoin. Specific diagnosis was established by detecting IgA serum activity to desmocollin 1 by indirect immunofluorescence microscopy on unfixed COS7 cells transfected with desmocollin 1. No IgA or IgG serum reactivity was found to recombinant forms of desmogleins 1 and 3 by an antigen-specific enzyme-linked immunosorbent assay. The disease was not effectively controlled by conventional therapeutic regimens. Systemic treatment with isotretinoin 20 mg daily led to complete clearance of skin lesions within 3 weeks. Assaying IgA serum reactivity to desmocollin 1, desmoglein 1, and desmoglein 3 as a valuable method for establishing the diagnosis and differentiating the 2 subtypes of IgA pemphigus. Isotretinoin was an effective drug in the treatment of subcorneal pustular dermatosis type of IgA pemphigus in this patient.

Original languageEnglish
Pages (from-to)923-926
Number of pages4
JournalJournal of the American Academy of Dermatology
Volume43
Issue number5 SPEC. SUPPL.
DOIs
Publication statusPublished - 2000 Jan 1
Externally publishedYes

ASJC Scopus subject areas

  • Dermatology

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    Gruss, C., Zillikens, D., Hashimoto, T., Amagai, M., Kroiss, M., Vogt, T., Landthaler, M., & Stolz, W. (2000). Rapid response of IgA pemphigus of subcorneal pustular dermatosis type to treatment with isotretinoin. Journal of the American Academy of Dermatology, 43(5 SPEC. SUPPL.), 923-926. https://doi.org/10.1067/mjd.2000.104002