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Dermatitis herpetiformis

Duhring disease

 

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

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 exact cause is unknown. It is an autoimmune disorder. There is a strong link between dermatitis herpetiformis and celiac disease. Celiac disease is an autoimmune disorder that causes inflammation in the small intestine from eating gluten. People with dermatitis herpetiformis also have a sensitivity to gluten, which causes the skin rash. About 25% of people with celiac disease also have dermatitis herpetiformis.

 

Symptoms

 

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 people may have scratch marks and skin erosions instead of blisters

Most people with dermatitis herpetiformis 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

 

Thyroid disease may be found in many people with dermatitis herpetiformis. People with dermatitis herpetiformis are also more likely to develop certain cancers of the intestines. The drugs used to treat dermatitis herpetiformis can also have side effects. Blood tests will be needed to check the levels of these drugs in the body.

 

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

Bolognia JL, Schaffer JV, Duncan KO, Ko CJ. Dermatitis herpetiformis and linear IgA bullous dermatosis. In: Bolognia JL, Schaffer JV, Duncan KO, Ko CJ, eds. Dermatology Essentials. 3rd ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 25.

Cardones AR, Hall RP III. Pathophysiology of dermatitis herpetiformis: a model for cutaneous manifestations of gastrointestinal inflammation. Immunol Allergy Clin North Am. 2012;32:263-74. PMID: 22560139 www.ncbi.nlm.nih.gov/pubmed/22560139.

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

Zone JJ. Dermatitis herpetiformis. In: Lebwohl MG, Heymann WR, Berth-Jones J, Coulson I, eds. Treatment of Skin Disease: Comprehensive Therapeutic Strategies. 4th ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 55.

 
  • 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

    Self Care

     

      Tests for Dermatitis herpetiformis

       

         

        Review Date: 4/14/2015

        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, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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