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Dermatitis herpetiformis

Definition

Dermatitis herpetiformis is an extremely itchy rash consisting of bumps and blisters. The rash is chronic, which means it continues over a long period.

Alternative Names

Duhring disease

Causes

Dermatitis herpetiformis usually begins in people age 20 and older. Children can sometimes be affected. It is seen in both men and women.

The cause is unknown. It is thought to be an autoimmune disorder. Dermatitis herpetiformis is also linked to gluten sensitivity (celiac sprue disease) in the small bowel.

Symptoms

Symptoms of dermatitis herpetiformis tend to come and go. Symptoms include:

  • Extremely itchy bumps or blisters, most often on the elbows, knees, back, and buttocks
  • The rash is usually the same size and shape on both sides
  • The rash can look like eczema
  • Some patients may have scratch marks instead of blisters

Exams and Tests

A skin biopsy and direct immunofluorescence test of the skin are performed in most cases. Your doctor may also recommend a biopsy of the intestines. Blood tests may be ordered to confirm the diagnosis.

Treatment

An antibiotic called dapsone is extremely effective.

A strict gluten-free diet will also be recommended to help control the disease. Sticking to this diet may eliminate the need for medications and prevent later complications.

Immunosuppressive medications may be used, but are less effective.

Outlook (Prognosis)

The disease may be well-controlled with treatment. Without treatment, there may be a significant risk of intestinal cancer.

Possible Complications

Thyroid disease may be found in many patients with dermatitis herpetiformis. Patients are also more likely to develop certain cancers of the intestines.

When to Contact a Medical Professional

Call your health care provider if you have a rash that continues despite home treatment.

Prevention

There is no known prevention of this disease. People with this condition may be able to prevent complications by avoiding foods that contain gluten.

References

Hull CM, Zone JJ. Dermatitis herpetiformis and linear IgA bullous dermatosis. In: Bolognia JL, Jorizzo JL, Schaffer JV, eds. Dermatology. 3rd ed. Philadelphia, Pa: Elsevier Saunders; 2012:chap 31.

Habif TP. Clinical Dermatology: A Color Guide to Diagnosis and Therapy. 5th ed. Philadelphia, Pa: Elsevier Mosby; 2009:chap 16.


Review Date: 5/15/2013
Reviewed By: Kevin Berman, MD, PhD, Atlanta Center for Dermatologic Disease, Atlanta, GA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Bethanne Black, and the A.D.A.M. Editorial team.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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