Dermatitis herpetiformis, also known as Duhring disease, is a rare, chronic, skin disorder characterized by the presence of groups of severely itchy (pruritic) blisters and raised skin lesions (papules). These are more common on the knees, elbows, buttocks and shoulder blades. The exact cause of this disease is not known although it is frequently associated with the inability to digest gluten (gluten sensitive enteropathy [GSE] or celiac sprue).
The slow onset of the symptoms of dermatitis herpetiformis usually begins during adulthood, but children can also be affected. Symptoms include the appearance on the skin of blisters, fluid-filled sores, red lesions that resemble hives, and/or well-defined, raised skin lesions (papules). The skin typically becomes intensely itchy and red. Itching and burning may be almost intolerable and the need to scratch may become irresistible. Skin lesions develop on both sides of the body, especially on the head, elbows, knees, lower back, and/or buttocks. Frequently people with dermatitis herpetiformis develop blisters and papules on the face and neck. Approximately fifty percent of people with Duhring disease have dark discolorations (pigmentation) in the areas where skin lesions are present.
Approximately seventy-five percent of people with dermatitis herpetiformis also have an increased sensitivity to gluten. Gluten is a protein that is present in wheat, oats, rye, barley, and millet. The symptoms associated with the loss of the ability to properly digest gluten (gluten-sensitive enteropathy) are similar to those of celiac disease. (For more information on celiac disease, see the Related Disorders section of this report.)
The exact cause of dermatitis herpetiformis is not fully understood. Several studies have suggested that there may be a genetic predisposition to this disease. A genetic predisposition means that a person may carry a gene for a disease but it may not be expressed unless something in the environment triggers the disease. Approximately ninety percent of people with this disease are positive for HLA-B8 gene, a marker that may indicate a predisposition to this disease.
Abnormalities of the immune system, especially the presence of IgA (immunoglobulin A) in skin lesions, are common in patients with dermatitis herpetiformis. Particular drugs, such as iodide and other halides can cause a flare-up of symptoms. The exact role of gluten as a cause of this disease is not understood.
Recently, the presence of epidermal transglutaminase has been detected in the skin of patients with dermatitis herpetiformis. Epidermal transglutaminase is very closely akin to tissue transglutaminase, the enzyme characteristic of celiac disease. Research continues to determine whether this finding may help to explain what causes dermatitis herpetiformis.
Dermatitis herpetiformis affects more males than females by a ratio of 3:2. It is estimated that this disorder affects as many as 39 cases per 100,000. Although the symptoms may begin at any age, this disease usually begins during middle adult life. It is very rare in children.
The diagnosis of dermatitis herpetiformis may be made by direct immunofluorescence testing on samples of skin tissue. This test is designed to demonstrate the presence of IgA, a protein antibody (immunoglobulin), not normally in skin tissue.
People with this disease are typically treated with the drug dapsone. This drug usually relieves the symptoms and improves the skin rash within 1 or 2 days. The urgent need to scratch usually subsides within 1 to 3 days. Since one of the most common side effects of dapsone is the destruction of red blood cells (hemolysis), people taking this medication must have periodic blood tests to monitor for any changes in their blood counts.
The application of a topical corticosteroid cream to affected areas on the skin may help to relieve itching in some people with this disease. However, the administration of oral nonsteroidal antiinflammatory drugs (NSAIDS) usually aggravates the symptoms of dermatitis herpetiformis.
Some people with dermatitis herpetiformis who also have gluten sensitive enteropathy may be able to discontinue drug therapy if they follow a strict gluten-free diet for at least 6 to 12 months. Gluten is found in all grains except corn and rice. Special gluten-free foods are commercially available.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.
For information about clinical trials being conducted at the National Institutes of Health (NIH) in Bethesda, MD, contact the NIH Patient Recruitment Office:
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Researchers are exploring the relationship between the skin and the function of the digestive system in people with dermatitis herpetiformis and other similar dermatological diseases. For more information, please contact:
Russell P. Hall, III, M.D.
Duke University Medical Center
Durham, NC 27710
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FROM THE INTERNET
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