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Dermatitis Herpetiformis

By

Daniel M. Peraza

, MD, Geisel School of Medicine at Dartmouth University

Last full review/revision Sep 2020| Content last modified Sep 2020
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Topic Resources

Dermatitis herpetiformis is an autoimmune disorder causing clusters of intensely itchy, red blisters and hivelike swellings in people who have celiac disease.

  • In this autoimmune disorder, glutens in wheat, rye, and barley products cause the immune system to attack the skin.

  • People have red, itchy blisters and hivelike swellings on various areas of the body.

  • Doctors diagnose dermatitis herpetiformis by examining skin samples under a microscope.

  • People usually respond to treatment with dapsone or sulfapyridine and a gluten-free diet.

The body's immune system Overview of the Immune System The immune system is designed to defend the body against foreign or dangerous invaders. Such invaders include Microorganisms (commonly called germs, such as bacteria, viruses, and fungi) Parasites... read more makes special cells that protect the body against harmful foreign invaders such as bacteria and viruses. Some of these cells respond to invaders by producing proteins called antibodies. Antibodies Antibodies One of the body's lines of defense (immune system) involves white blood cells (leukocytes) that travel through the bloodstream and into tissues, searching for and attacking microorganisms and... read more Antibodies target and attach to the invaders and attract other cells in the immune system to destroy them. In an autoimmune disorder Autoimmune Disorders An autoimmune disorder is a malfunction of the body's immune system that causes the body to attack its own tissues. What triggers autoimmune disorders is not known. Symptoms vary depending on... read more , the body’s immune system mistakenly attacks the body’s own tissues—in this case, the skin. The antibodies that are produced mistakenly target the tissues as invaders, which makes them susceptible to destruction.

Dermatitis herpetiformis often occurs in young adults but can occur in children and older people. It is rare in blacks and Asians.

Despite its name, dermatitis herpetiformis has nothing to do with the herpesvirus. The term herpetiformis is used to describe the way in which the blisters cluster together (similar to a rash caused by some herpesviruses).

In people with dermatitis herpetiformis, glutens (proteins) in wheat, rye, and barley products somehow activate the immune system, which attacks parts of the skin and causes the rash and itching. People with dermatitis herpetiformis often have celiac disease Celiac Disease Celiac disease is a hereditary intolerance to gluten (a protein found in wheat, barley, and rye) that causes characteristic changes in the lining of the small intestine, resulting in malabsorption... read more Celiac Disease , which is an intestinal disorder caused by sensitivity to gluten, but they may not have symptoms resulting from the celiac disease. People also have a higher incidence of other autoimmune disorders, such as thyroiditis Hashimoto Thyroiditis Hashimoto thyroiditis is chronic, autoimmune inflammation of the thyroid. Hashimoto thyroiditis results when the body attacks the cells of the thyroid gland—an autoimmune reaction. At first... read more , systemic lupus erythematosus Systemic Lupus Erythematosus (SLE) Systemic lupus erythematosus is a chronic autoimmune inflammatory connective tissue disorder that can involve joints, kidneys, skin, mucous membranes, and blood vessel walls. Problems in the... read more Systemic Lupus Erythematosus (SLE) , sarcoidosis Sarcoidosis Sarcoidosis is a disease in which abnormal collections of inflammatory cells (granulomas) form in many organs of the body. Sarcoidosis usually develops in people aged 20 to 40, most often people... read more Sarcoidosis , pernicious anemia Vitamin B12 Deficiency Vitamin B12 deficiency can occur in vegans who do not take supplements or as a result of an absorption disorder. Anemia develops, causing paleness, weakness, fatigue, and, if severe, shortness... read more , and diabetes Diabetes Mellitus (DM) and Disorders of Blood Sugar Metabolism read more . People with dermatitis herpetiformis occasionally develop lymphoma in the intestines Small-Intestine Cancer Blood in the stool is a common symptom, but sometimes the cancer blocks the intestine, causing crampy abdominal pain and vomiting. The diagnosis is based on various intestinal viewing techniques... read more .

Symptoms of Dermatitis Herpetiformis

The blisters and hives usually develop gradually, mostly on the elbows, knees, buttocks, lower back, and back of the head, but can develop suddenly. Sometimes blisters break out on the face and neck. Itching and burning are likely to be severe. Because itching is intense and the skin is fragile, blisters usually break quickly, and few remain intact for the doctor to see. Blisters may develop in the mouth but they usually do not cause symptoms.

Iodides and iodine-containing preparations (such as kelp and seaweed products and certain skin cleansers) may worsen the rash. Some specialists also suggest avoiding iodized salt.

Did You Know...

  • Dermatitis herpetiformis is not related to the herpesvirus.

Diagnosis of Dermatitis Herpetiformis

Treatment of Dermatitis Herpetiformis

  • Gluten-free diet

  • Dapsone and sometimes other drugs

The drug dapsone, taken by mouth, almost always provides relief in 1 to 3 days but requires that blood counts be checked regularly because dapsone can cause anemia. Sulfapyridine (or, alternatively, sulfasalazine) is also taken by mouth and may be given to people who cannot tolerate dapsone. However, sulfapyridine can cause anemia and low numbers of white blood cells (increasing the risk of infection) and also requires that blood counts be checked regularly.

Once the disease has been brought under control with drugs and people have followed a strict gluten-free diet, drug treatment can sometimes be discontinued. However, some people can never stop taking the drugs. In most people, any re-exposure to gluten, however small, triggers another outbreak. A gluten-free diet that is strictly followed for 5 to 10 years decreases the risk of intestinal lymphoma Small-Intestine Cancer Blood in the stool is a common symptom, but sometimes the cancer blocks the intestine, causing crampy abdominal pain and vomiting. The diagnosis is based on various intestinal viewing techniques... read more .

More Information

The following is an English-language resource that may be useful. Please note that THE MANUAL is not responsible for the content of this resource.

Drugs Mentioned In This Article

Generic Name Select Brand Names
AZULFIDINE
ACZONE
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Dermatitis Herpetiformis
Dermatitis herpetiformis is an autoimmune disease that causes clusters of intensely itchy small blisters and hive-like swellings on the skin of people who have celiac disease. Which of the following foods can trigger an attack of dermatitis herpetiformis? 
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