Dermatitis Herpetiformis is an uncommon, chronic disease in which the primary clinical manifestations are cutaneous (relating to the skin). It is also known as Brocq-During disease, dermatitis multiformis, Duhring disease, and gluten-sensitive enteropathy.
It usually occurs in association with an asymptomatic gluten-sensitive enteropathy (disease of the intestine, similar to that in celiac disease.
Celiac disease is caused by a sensitivity to gluten, a protein found in wheat, oats, rye, and barley. The intolerance to gluten causes the lining of the intestine to lose its tiny folds (villi) through which nutrients are absorbed. In addition, digestive enzymes are no longer produced in the intestinal lining in adequate amounts. The common symptoms are foul-smelling diarrhea, bloated abdomen, and anemia.
Dermatitis herpetiformis is idiopathic (without known cause), but several immunologic abnormalities have been detected. The presence in lesions of IgA (immunoglobulin) and complement components supports an immunoregulatory disturbance. Iodide and other halides may cause a flare.
Onset may be at any age, but it usually occurs in middle adult life. A slow onset during adulthood is typical, but children can be affected. Males are affected more than females.
It appears to have its highest prevalence in Scandinavia.
Small blisters, discrete papules (bumps), and itchy, smooth lesions resembling hives appear symmetrically on the head, elbows, knees, lower back, and buttocks. Quite often, blisters and papules occur on the face and neck. Itching and burning may be almost intolerable, and the need to scratch irresistible.
Ingestion of gluten plays a role in the exacerbation of skin lesions.
Diagnosis is made by physical findings and microscopic analysis of a skin biopsy.
The gluten-free diet is seriously restrictive and compliance may be difficult. Foods with gluten include wheat, oats, rye, and barley. Difficulties in diet stem from gluten being accidentally ingested from time to time. Many patients also report great difficulty in eating away from home and in determining whether a food is gluten-free.
Standard treatment frequently involves the use of dapsone to treat the rash. This brings symptomatic relief within 1 or 2 days. The urgent need to scratch usually abates in 1 to 3 days.
Dapsone can be associated with severe hematologic (blood) disturbances and must be closely monitored. However, strict long-term avoidance of dietary gluten has been shown to reduce the dose of dapsone required to control the disease and may even eliminate the need for medication. In addition, a gluten-free diet may reduce the risk of gastrointestinal lymphoma.