Dermatitis herpetiformis is an autoimmune disorder causing clusters of intensely itchy, red blisters and hivelike swellings in people who have celiac disease.

In this autoimmune disorder, glutens in wheat, rye, and barley products cause the immune system to attack the skin.

People have red, itchy blisters and hivelike swellings on various areas of the body.

Doctors diagnose dermatitis herpetiformis by examining skin samples under a microscope.

People usually respond to treatment with dapsone or sulfapyridine and a gluten-free diet.

The body''s immune system makes special cells that protect the body against harmful foreign invaders such as bacteria and viruses. Some of these cells respond to invaders by producing proteins called antibodies. Antibodies target and attach to the invaders and attract other cells in the immune system to destroy them. In an autoimmune disorder, the body’s immune system mistakenly attacks the body’s own tissues—in this case, the skin. The antibodies that are produced mistakenly target the tissues as invaders, which makes them susceptible to destruction.

Dermatitis herpetiformis affects people of all ages but is most commonly diagnosed in adults between the ages of 30 to 40. It is rare in Black and Asian people.

Despite its name, dermatitis herpetiformis has nothing to do with the herpesvirus. The term herpetiformis is used to describe the way in which the blisters cluster together (similar to a rash caused by some herpesviruses).

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 often have celiac disease, which is an intestinal disorder caused by sensitivity to gluten, but they may not have symptoms resulting from the celiac disease. People also have a higher incidence of other autoimmune disorders, such as thyroiditis, systemic lupus erythematosus, sarcoidosis, pernicious anemia, and diabetes. People with dermatitis herpetiformis occasionally develop lymphoma in the intestines.