Section 18. Skin Disorders

Chapter 209. Blistering Diseases

Topics: Introduction | Pemphigus | Bullous Pemphigoid | Dermatitis Herpetiformis

Dermatitis Herpetiformis

Dermatitis herpetiformis is an autoimmune disease causing clusters of intensely itchy small blisters and hivelike swellings.

Despite its name, dermatitis herpetiformis has nothing to do with the herpes virus. In people with dermatitis herpetiformis, glutens (proteins) in wheat, rye, and barley products somehow activate the immune system, which attacks parts of the skin and causes the rash and itching. People with dermatitis herpetiformis may develop celiac disease (see Section 9, Chapter 125), which is caused by the gluten sensitivity. These people have a higher incidence of other autoimmune diseases, such as thyroiditis, systemic lupus erythematosus, sarcoidosis, and diabetes. People with dermatitis herpetiformis occasionally develop lymphoma in the intestines.

Small blisters usually develop gradually, mostly on the elbows, knees, buttocks, lower back, and back of the head. Sometimes blisters break out on the face and neck. Itching and burning are likely to be severe. Anti-inflammatory drugs, such as ibuprofen, may worsen the rash.

Diagnosis and Treatment

The diagnosis is based on a skin biopsy, in which doctors find particular kinds and patterns of antibodies in the skin samples.

The blisters do not go away without treatment. The drug dapsone, taken by mouth, almost always provides relief in 1 to 2 days, but requires that blood counts be checked regularly. Once the disease has been brought under control with drugs and the person has followed a strict gluten-free diet (a diet that is free of wheat, rye, and barley) for 6 months or longer, drug treatment usually can be discontinued. However, some people can never discontinue the drug. In most people, any reexposure to gluten, however small, will trigger another outbreak. A gluten-free diet may prevent the development of intestinal lymphoma.