title: Rapid response of IgA pemphigus of subcorneal pustular dermatosis type to treatment with isotretinoin.
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.
authors.affiliation: Department of Dermatology, University of Regensburg, Franz-Josef-Strauss-Allee 11, D-93042 Regensburg, Germany. claus.gruss@klinik.uni-regensburg.de
authors.forename: C
authors.initials: C
authors.lastname: Gruss
authors.forename: D
authors.initials: D
authors.lastname: Zillikens
authors.forename: T
authors.initials: T
authors.lastname: Hashimoto
authors.forename: M
authors.initials: M
authors.lastname: Amagai
authors.forename: M
authors.initials: M
authors.lastname: Kroiss
authors.forename: T
authors.initials: T
authors.lastname: Vogt
authors.forename: M
authors.initials: M
authors.lastname: Landthaler
authors.forename: W
authors.initials: W
authors.lastname: Stolz
mesh_terms: D000368:Aged; D015815:Cell Adhesion Molecules / Q000276:immunology; D003598:Cytoskeletal Proteins / Q000276:immunology; D003879:Dermatologic Agents / Q000627:therapeutic use*; D051187:Desmocollins; D051183:Desmoglein 1; D051184:Desmoglein 3; D051182:Desmogleins; D051180:Desmoplakins; D003937:Diagnosis, Differential; D005455:Fluorescent Antibody Technique; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis* / Q000097:blood; D015474:Isotretinoin / Q000627:therapeutic use*; D008297:Male; D010392:Pemphigus / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D012867:Skin / Q000276:immunology / Q000473:pathology
chemical_list: D015815:Cell Adhesion Molecules; D003598:Cytoskeletal Proteins; C495297:DSG1 protein, human; D003879:Dermatologic Agents; D051187:Desmocollins; D051183:Desmoglein 1; D051184:Desmoglein 3; D051182:Desmogleins; D051180:Desmoplakins; D007070:Immunoglobulin A; D015474:Isotretinoin