Primary biliary cirrhosis associated with coeliac disease and dermatitis herpetiformis

A 69-year-old man who 1 year previously got the diagnosis primary biliary cirrhosis presented a highly pruritic rash on the knees, elbows and in the inguinal region. Both clinically and histologically this was compatible with dermatitis herpetiformis, and dapsone treatment confirmed this. He was found to have jejunal atrophy, and after 1 year with gluten-free diet, biopsy showed almost full restoration of the villous pattern. He then only rarely had any skin problems.