title: IgA pemphigus: the first two Scandinavian cases.
abstract: IgA pemphigus is a rare neutrophilic acantholytic skin disorder with only approximately 70 cases reported in the indexed literature to date. Here we describe two patients with IgA pemphigus (subcorneal pustular dermatosis type and intraepithelial neutrophilic type) that to our knowledge are the first Scandinavian patients with this disease. Initially, both patients were misdiagnosed as subcorneal pustular dermatosis of Sneddon and Wilkinson and only subsequent careful immunofluorescence studies (in one case with confocal laser scanning microscopy) led to the correct diagnosis. Apart from the expected IgA depositions on epidermal cell surfaces, both patients demonstrated some degree of intercellular IgG-specific immunofluorescence. No circulating IgA autoantibodies were detected. One patient was treated with the standard regime comprising dapsone and prednisolone, whereas in the other case a novel methotrexate and prednisolone combination treatment showed marked clinical efficacy.
authors.affiliation: Department of Dermatology, Bispebjerg Hospital, Copenhagen, Denmark. rgniadecki@hotmail.com
authors.forename: Robert
authors.initials: R
authors.lastname: Gniadecki
authors.forename: Anette
authors.initials: A
authors.lastname: Bygum
authors.forename: Ole
authors.initials: O
authors.lastname: Clemmensen
authors.forename: Else
authors.initials: E
authors.lastname: Svejgaard
authors.forename: Susanne
authors.initials: S
authors.lastname: Ullman
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001768:Blister / Q000188:drug therapy / Q000276:immunology; D003622:Dapsone / Q000627:therapeutic use; D003879:Dermatologic Agents / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D019085:Fluorescent Antibody Technique, Direct; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis*; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D018613:Microscopy, Confocal; D008875:Middle Aged; D010392:Pemphigus / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D011239:Prednisolone / Q000627:therapeutic use; D012537:Scandinavian and Nordic Countries; D012867:Skin / Q000276:immunology / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D003879:Dermatologic Agents; D007070:Immunoglobulin A; D003622:Dapsone; D011239:Prednisolone; D008727:Methotrexate