Mobacken H, Kastrup W, Ljunghall K, Löfberg H, Nilsson LA, Svensson A, Tjernlund U.
DOI: 10.2340/0001555563123128

Ten adult patients with homogeneous-linear deposition of IgA along the basement membrane zone have been studied. The direct immunofluorescence IgA pattern was stable, and there was no deposition of IgG or IgM. The clinical presentations were heterogeneous and resembled dermatitis herpetiformis (DH) (3 cases) or bullous pemphigoid (5 cases). Two patients had widespread gyrate blistering lesions of acute onset. Pruritus was constantly present. The course of the disease was chronic except for one patient who had a spontaneous remission after 5 years. The histology was indistinguishable from "classical" DH with granular IgA in dermal papillae. The patients studied in the present investigation did not show the high incidence of atrophic intestinal villi found in "classical" DH. Five of 9 cases carried the haplotype HLA-A1, B8, DR3. In spite of a close similarity between linear IgA dermatosis and DH, differences exist which indicate discrepancies in etiopathogenesis.