Dermatitis herpetiformis Duhring

Dermatitis herpetiformis Duhring is a chronic, blistering autoimmune disease of the skin that is closely associated with coeliac disease. It affects around 10 to 20 percent of people with gluten intolerance and usually occurs between the ages of 30 and 40, with men being affected more frequently than women. The disease is caused by an immune reaction against gluten, a protein found in wheat, rye and barley.

Causes

The body produces antibodies which are deposited in the skin and trigger an inflammatory reaction. The disease is characterized by very itchy, burning blisters and papules that are arranged symmetrically. Preferred sites are the extensor sides of the elbows and knees, buttocks, shoulders, neck and scalp.

Symptoms

The itching is often so severe that the blisters are scratched open before they are really visible. This results in crusts and scratch marks. The symptoms occur in episodes and can persist for years.

Diagnosis

Diagnosis is made by taking a skin biopsy from healthy skin next to an affected area. Direct immunofluorescence shows typical deposits of IgA antibodies on the basement membrane. In addition, blood tests for specific antibodies and a biopsy of the small intestine are carried out to detect coeliac disease.

Treatment

The most important treatment is a lifelong, strictly gluten-free diet. This usually leads to a significant improvement in the skin symptoms after months and also prevents the intestinal damage caused by coeliac disease. The drug dapsone, which effectively suppresses itching and blistering, is used for rapid symptom relief. The prognosis is good with a consistent gluten-free diet. Without treatment, there is an increased risk of lymphoma and other complications of coeliac disease.