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.
Autoimmune disorder
An autoimmune disorder occurs when the body's immune system attacks and destroys healthy body tissue by mistake. There are more than 80 types of aut...
Celiac disease
Celiac disease is a condition caused by damage to the lining of the small intestine. This damage comes from a reaction to eating gluten. This is a ...
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.
Skin biopsy
A skin lesion biopsy is when a small amount of skin is removed so it can be examined. The skin is tested to look for skin conditions or diseases. A...
Biopsy
A biopsy is the removal of a small piece of tissue for laboratory examination.
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.
Gluten-free diet
Celiac disease is an immune disorder passed down through families. Gluten is a protein found in wheat, barley, rye, or sometimes oats. It may also b...
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.
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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
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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
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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
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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
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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
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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
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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 - 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 - 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 - 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 - 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 - 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: 4/14/2015
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.