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Dermatitis herpetiformis

Duhring disease; DH

Dermatitis herpetiformis (DH) is a very itchy rash consisting of bumps and blisters. The rash is chronic (long-term).

Causes

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

The exact cause is unknown. Despite the name, it is not related to the herpes virus. DH is an autoimmune disorder. There is a strong link between DH and celiac disease. Celiac disease is an autoimmune disorder that causes inflammation in the small intestine from eating gluten. People with DH also have a sensitivity to gluten, which causes the skin rash. About 25% of people with celiac disease also have DH.

Symptoms

Symptoms include:

  • Extremely itchy bumps or blisters, most often on the elbows, knees, back, and buttocks.
  • Rashes that are usually the same size and shape on both sides.
  • The rash can look like eczema.
  • Scratch marks and skin erosions instead of blisters in some people. 

Most people with DH have damage to their intestines from eating gluten. But only some have intestinal symptoms.

Exams and Tests

In most cases, a skin biopsy and direct immunofluorescence test of the skin are performed. The health care provider may also recommend a biopsy of the intestines. Blood tests may be ordered to confirm the diagnosis.

Treatment

An antibiotic called dapsone is very effective.

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

Drugs that supress the immune system 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

Complications may include:

  • Autoimmune thyroid disease
  • Develop certain cancers, especially lymphomas of the intestines
  • Side effects of the drugs used to treat DH

When to Contact a Medical Professional

Call your provider if you have a rash that continues despite 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, Schaffer JV, Cerroni L, eds. Dermatology. 4th ed. Philadelphia, PA: Elsevier; 2018:chap 31.

Kelly CP. Celiac disease. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology/Diagnosis/Management. 10th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 107.

    • Dermatitis, herpetiformis - close-up of lesion

      Dermatitis, herpetiformis - close-up of lesion - illustration

      Dermatitis herpetiformis is a chronic inflammatory disease that produces lesions that burn and itch intensely. This is a close-up of dermatitis herpetiformis lesions. The lesions are red (erythematous) and may be slightly raised (papular), form small pus-filled areas (pustules), or there may be blisters (vesicles). The disease develops suddenly and may last from weeks to months. It may occur in association with gluten (wheat) sensitivity and allergy.

      Dermatitis, herpetiformis - close-up of lesion

      illustration

    • Dermatitis - herpetiformis on the knee

      Dermatitis - herpetiformis on the knee - illustration

      This picture shows the knee of a person with a chronic inflammatory disease known as dermatitis herpetiformis. It produces red, raised (papular), small or large blisters (vesicles or bullae) that burn and itch intensely. Dermatitis herpetiformis develops suddenly, lasts for weeks to months, and may be associated with digestive diseases (such as Celiac disease).

      Dermatitis - herpetiformis on the knee

      illustration

    • Dermatitis - herpetiformis on the arm and legs

      Dermatitis - herpetiformis on the arm and legs - illustration

      This picture shows a chronic inflammatory disease (dermatitis herpetiformis) that produces red (erythematous), raised (papular), small or large blisters (vesicles or bullae) that burn and itch intensely. Dermatitis herpetiformis develops suddenly, lasts for weeks to months, and may be associated with digestive diseases (such as Celiac disease).

      Dermatitis - herpetiformis on the arm and legs

      illustration

    • Dermatitis herpetiformis on the thumb

      Dermatitis herpetiformis on the thumb - illustration

      This picture shows the thumb of a person with a chronic inflammatory disease known as dermatitis herpetiformis. It produces red, raised (papular), small or large blisters (vesicles or bullae) that burn and itch intensely. Dermatitis herpetiformis develops suddenly, lasts for weeks to months, and may be associated with digestive diseases such as celiac disease.

      Dermatitis herpetiformis on the thumb

      illustration

    • Dermatitis herpetiformis on the hand

      Dermatitis herpetiformis on the hand - illustration

      This picture shows the fingers of a person with a chronic inflammatory disease known as dermatitis herpetiformis. It produces red, raised (papular), small or large blisters (vesicles or bullae) that burn and itch intensely. Dermatitis herpetiformis develops suddenly, lasts for weeks to months, and may be associated with digestive diseases such as celiac disease.

      Dermatitis herpetiformis on the hand

      illustration

    • Dermatitis herpetiformis on the forearm

      Dermatitis herpetiformis on the forearm - illustration

      This picture shows the forearm of a person with a chronic inflammatory disease known as dermatitis herpetiformis. It produces red, raised (papular), small or large blisters (vesicles or bullae) that burn and itch intensely. Dermatitis herpetiformis develops suddenly, lasts for weeks to months, and may be associated with digestive diseases such as celiac disease.

      Dermatitis herpetiformis on the forearm

      illustration

      • Dermatitis, herpetiformis - close-up of lesion

        Dermatitis, herpetiformis - close-up of lesion - illustration

        Dermatitis herpetiformis is a chronic inflammatory disease that produces lesions that burn and itch intensely. This is a close-up of dermatitis herpetiformis lesions. The lesions are red (erythematous) and may be slightly raised (papular), form small pus-filled areas (pustules), or there may be blisters (vesicles). The disease develops suddenly and may last from weeks to months. It may occur in association with gluten (wheat) sensitivity and allergy.

        Dermatitis, herpetiformis - close-up of lesion

        illustration

      • Dermatitis - herpetiformis on the knee

        Dermatitis - herpetiformis on the knee - illustration

        This picture shows the knee of a person with a chronic inflammatory disease known as dermatitis herpetiformis. It produces red, raised (papular), small or large blisters (vesicles or bullae) that burn and itch intensely. Dermatitis herpetiformis develops suddenly, lasts for weeks to months, and may be associated with digestive diseases (such as Celiac disease).

        Dermatitis - herpetiformis on the knee

        illustration

      • Dermatitis - herpetiformis on the arm and legs

        Dermatitis - herpetiformis on the arm and legs - illustration

        This picture shows a chronic inflammatory disease (dermatitis herpetiformis) that produces red (erythematous), raised (papular), small or large blisters (vesicles or bullae) that burn and itch intensely. Dermatitis herpetiformis develops suddenly, lasts for weeks to months, and may be associated with digestive diseases (such as Celiac disease).

        Dermatitis - herpetiformis on the arm and legs

        illustration

      • Dermatitis herpetiformis on the thumb

        Dermatitis herpetiformis on the thumb - illustration

        This picture shows the thumb of a person with a chronic inflammatory disease known as dermatitis herpetiformis. It produces red, raised (papular), small or large blisters (vesicles or bullae) that burn and itch intensely. Dermatitis herpetiformis develops suddenly, lasts for weeks to months, and may be associated with digestive diseases such as celiac disease.

        Dermatitis herpetiformis on the thumb

        illustration

      • Dermatitis herpetiformis on the hand

        Dermatitis herpetiformis on the hand - illustration

        This picture shows the fingers of a person with a chronic inflammatory disease known as dermatitis herpetiformis. It produces red, raised (papular), small or large blisters (vesicles or bullae) that burn and itch intensely. Dermatitis herpetiformis develops suddenly, lasts for weeks to months, and may be associated with digestive diseases such as celiac disease.

        Dermatitis herpetiformis on the hand

        illustration

      • Dermatitis herpetiformis on the forearm

        Dermatitis herpetiformis on the forearm - illustration

        This picture shows the forearm of a person with a chronic inflammatory disease known as dermatitis herpetiformis. It produces red, raised (papular), small or large blisters (vesicles or bullae) that burn and itch intensely. Dermatitis herpetiformis develops suddenly, lasts for weeks to months, and may be associated with digestive diseases such as celiac disease.

        Dermatitis herpetiformis on the forearm

        illustration

      Review Date: 4/16/2019

      Reviewed By: Michael Lehrer, MD, Clinical Associate Professor, Department of Dermatology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, 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. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., a business unit of Ebix, Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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