FAMILIAL DERMATITIS-HERPETIFORMIS

The possibility that dermatitis herpetiformis (DH) might have a genetic background had been set forth during the past 20 years. Before that only a few familial cases were reported (1-4), and several surveys of DH patients do not mention or did not find affected relatives (5-7). When DH was linked in 1966 to celiac disease (8), a known familial disease with 10 to 20% of affected first-degree relatives (9,10), it seemed natural to carry out similar surveys on relatives of patients with DH. Marks et al (11) performed jejunal biopsies on 18 relatives and showed that 7 (40%) had findings consistent with gluten-sensitive enteropathy. Reunala et al (12) investigated the families of six DH patients and found in their relatives a similar high frequency of jejunal villous atrophy. The enteropathy was often subclinical and this seems to be one reason why celiac disease has previously escaped the diagnosis in the relatives of DH patients. At present it is well documented that both in adults and in children the clinical spectrum of celiac disease can vary from the overt gastrointestinal disease with severe malabsorption to totally asymptomatic disease (13,14). Despite the increasing knowledge among dermatologists that DH has an immunogenetic background similar to that of celiac disease (CD), it is curious that only a few reports exist on familial DH. © 1991.