NORD gratefully acknowledges Sarah Zhou, MD Candidate, McGill University School of Medicine, and Christopher M. Hull, MD, Department of Dermatology University of Utah, for assistance in the preparation of this report.
Dermatitis herpetiformis (DH) is a rare, chronic, autoimmune skin condition characterized by the presence of groups of severely itchy blisters and raised red skin lesions. These are most commonly located on the elbows, knees, buttocks, lower back and scalp. The most common age of onset of DH is 30-40 years of age but individuals of all ages can be affected. DH is rare in children.
DH is a skin manifestation of celiac disease (CD). DH is treated with a gluten free diet and frequently a medication called dapsone.
This autoimmune disease may cause skin, oral and/or gastrointestinal manifestations related to gluten-sensitivity.
The classic finding of DH is the development of intensely itchy blisters, and red raised skin lesions that occur in groups. Itching and burning may be almost intolerable and the need to scratch may become irresistible. The most common sites for lesion development are the elbows, knees, buttocks, and scalp. The face and groin are less frequently involved.
Tooth enamel defects found in CD such as horizontal grooves, pits, or discoloration may occur in patients with DH. Patients with DH can rarely develop oral ulcerations (canker sores).
DH is a skin manifestation of CD and virtually every patient has gluten sensitivity causing inflammation and damage to the small intestine. DH patients generally have less gastrointestinal symptoms than patients with CD. Symptomatic patients may present with abdominal bloating, cramping, pain, diarrhea, or constipation.
The cause of DH appears to be complex, involving both genetic and environmental factors. The importance of gluten in the development of DH is supported by the observation that elimination of dietary gluten can result in remission of the lesions.
A genetic contribution to DH is supported by familial studies that demonstrate that first-degree relatives of patients with DH have an increased risk for both DH and CD. The genes that have been found to be closely associated with DH are certain HLA types (HLA-DQ2 and to a lesser extent, HLA-DQ8).
The main environmental factor that leads to DH is dietary gluten.
DH most frequently occurs in individuals of northern European heritage. It is estimated that this disorder affects as many as 75.3 per 100,000 people in northern Europe.
The incidence of DH appears to be decreasing, possibly due to better and earlier detection of CD (before they develop DH later in life).
The reported mean age of onset is in the third or fourth decade. However, individuals of all ages can be affected. It is uncommon in children.
Males have been reported to be slightly more likely to develop DH than females, but the reason for male predominance in DH is unknown.
In addition to recognizing the consistent clinical findings (see signs and symptoms), diagnosis of DH can be made by performing a skin biopsy for direct immunofluorescence microscopy (DIF). The characteristic finding that is necessary is the presence of antibody protein (IgA) deposits within the dermis. These antibody protein (IgA) deposits are not normally found in skin tissue. It has been reported that DIF is positive in DH 92% of the time.
Clinical Testing and Work-Up
The initial approach to diagnosis of DH who present with suggestive clinical findings involves obtaining a skin biopsy of the lesion for routine staining and a perilesional skin biopsy for DIF. Blood tests (serology) are used as an adjunctive test to support the diagnosis and can also be used to monitor adherence to dietary therapy.
The antibiotic dapsone is usually very effective in treating this condition. Symptomatic improvement may occur in as little as several hours after the first dose. However, as it may cause side effects, treatment with dapsone requires regular monitoring by a physician and regular laboratory testing.
A strict gluten-free diet is effective for both DH and the associated gastrointestinal manifestations. However, the benefits of a gluten-free diet may require several months to become noticeable. Strict adherence to a gluten-free diet may require counseling with a dietician to help identify and eliminate both obvious and hidden sources of dietary gluten, while also finding alternatives to gluten-containing foods.
The preferred approach to the treatment of DH is a combination of dapsone and a strict gluten-free diet. Dapsone is initially prescribed to achieve rapid clearance of symptoms. Tapering of the dapsone dose can often be achieved if patients maintain a strict gluten free diet. Many people will require some dose of dapsone to prevent flares which are usually related to inadvertent gluten intake.
For patients intolerant to dapsone, other medications may be used. The most common alternative is sulfapyridine. Topical corticosteroids may be used short-term to lessen the intensity of the itching, but do not suffice as long-term treatment.
Patients are usually managed by a dermatologist (for management of DH) and a nutritionist (for dietary counseling).
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