A Japanese case of ulcerative colitis associated with dermatitis herpetiformis and primary sclerosing cholangitis

A number of extraintestinal complications of ulcerative colitis have been described. However, only a few cases of ulcerative colitis associated with dermatitis herpetiformis have been documented. We report a case of ulcerative colitis associated with dermatitis herpetiformis and primary sclerosing cholangitis in a Japanese woman. The patient first developed exanthema on the extremities at the age of 18 years. Subsequently, her skin disease was diagnosed as dermatitis herpeti formis with linear IgA deposits at the dermoepidermal junction. A biopsy specimen from the jejunum did not show villous atrophy. Subsequently, she was diagnosed with primary sclerosing cholangitis at age 34 years, and with ulcerative colitis 2 years later. The patient died of hepatic failure at age 40 years. Bile duct cancer was found at autopsy. Both dermatitis herpetiformis and primary sclerosing cholangitis are diseases of the immune system that are associated with HLA-B8 and -DR3 in whites. Our patient had neither of these serotypes. The immune mechanisms involved in the extraintestinal complications of inflammatory bowel disease are briefly discussed.