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Gruss, C. et al.

Gruss, Zillikens, Hashimoto, Amagai, Kroiss, Vogt, Landthaler, Stolz,

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

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.[1]

References

Rapid response of IgA pemphigus of subcorneal pustular dermatosis type to treatment with isotretinoin. Gruss, C., Zillikens, D., Hashimoto, T., Amagai, M., Kroiss, M., Vogt, T., Landthaler, M., Stolz, W. J. Am. Acad. Dermatol. (2000) [Pubmed]

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