Desquamative gingivitis and balanitis--linear IgA disease or cicatricial pemphigoid?
A 38-year-old man presented with gingival inflammation together with erosions of the penis. Direct immunofluorescence demonstrated linear deposits of IgA at the basement membrane zone; indirect immunofluorescence and immunoblotting were negative. Linear IgA disease (LAD) was therefore suspected and treatment with dapsone initiated but this was changed to sulfamethoxy-pyridazine and systemic corticosteroids because of methaemoglobinaemia. During 1-year follow-up the lesions continued to wax and wane although they were never as extensive as before. Eighteen months after disease onset there was scarring of the penis together with suspected fibrosis of the inflamed gingival region. In addition the patient was HLA DQ7(3) positive, a haplotype thought to be increased in patients with cicatricial pemphigoid (CP); LAD with scarring or CP with solely linear IgA deposits are possible diagnoses of his condition.
Kirtschig
G
G
Department of Dermatology, Philipps-University Marburg, Germany.
Mengel
R
R
Mittag
H
H
Flores-De-Jacoby
L
L
Happle
R
R
D000328:Adult; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000188:drug therapy; D001446:Balanitis / Q000188:drug therapy / Q000209:etiology*; D003937:Diagnosis, Differential; D005891:Gingivitis / Q000188:drug therapy / Q000209:etiology*; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis*; D008297:Male; D010390:Pemphigoid, Benign Mucous Membrane / Q000175:diagnosis* / Q000188:drug therapy
D007070:Immunoglobulin A