Duhring disease; DH
Dermatitis herpetiformis (DH) is a very itchy rash consisting of bumps and blisters. The rash is chronic (long-term).
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.
- 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.
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.
The disease may be well-controlled with treatment. Without treatment, there may be a significant risk of intestinal cancer.
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.
There is no known prevention of this disease. People with this condition may be able to prevent complications by avoiding foods that contain gluten.
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.
Last reviewed on: 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.
What Dermatitis Herpetiformis Looks Like
Dermatitis Herpetiformis on the Hands
It's somewhat unusual to see dermatitis herpetiformis on someone's hands, but the rash can occur anywhere on the body.
Dermatitis herpetiformis may be more common in men (unlike celiac disease, which is diagnosed more often in women). In fact, some studies show a male-to-female ratio of up to two-to-one in dermatitis herpetiformis patients.
There's been comparably little research done on the health risks associated with dermatitis herpetiformis, but one study does show a risk of thinning bones that's comparable with the risk faced by those with celiac disease.
Another study found an increased risk for thyroid disease in those with dermatitis herpetiformis. This shouldn't be surprising since celiac disease and thyroid disease often are found together.
Frequently Asked Questions
How long does it take to develop a rash after you eat gluten?
Breakouts can occur within hours or days after eating gluten if you have celiac disease. If you have a wheat allergy, a rash might begin within two hours of eating.
Can I get a gluten rash if I don’t have celiac disease?
Yes. If you’re allergic to gluten (which is different than having celiac disease), you may develop an allergic rash.
Does celiac disease affect your eyes?
Possibly. In rare instances, celiac seems to affect different senses and could cause ophthalmic complications. These may result from people with celiac absorbing too little vitamin A, vitamin D, and calcium, or they may be due to related autoimmune disorders.
A Word From Verywell
Controlling your dermatitis herpetiformis through the gluten-free diet can be surprisingly difficult (you actually have to be significantly stricter to avoid skin symptoms than you do to avoid digestive symptoms from gluten). Anecdotally, those who have done it say it's worth it to eliminate the constant itching.
If you can get your rash under control and into remission, any future outbreaks should be less severe (and eventually, not even particularly noticeable).
Celiac Disease Doctor Discussion Guide
Get our printable guide for your next healthcare provider's appointment to help you ask the right questions.
Dermatitis Herpetiformis and Gluten Intolerance
What is dermatitis herpetiformis?
An itchy, blistering, burning skin rash, dermatitis herpetiformis (DH) is a difficult condition to live with. The rash and itching occur on the elbows, knees, scalp, back, and buttocks. This rash likely indicates gluten intolerance, which may be related to a more serious underlying condition known as celiac disease. DH is sometimes called Duhring’s disease or gluten rash. People who have this condition need to maintain a strict gluten-free diet.
What causes dermatitis herpetiformis?
From the sound of the name, many people think this rash is caused by some form of the herpes virus. This isn’t the case, as it has nothing to do with herpes. Dermatitis herpetiformis occurs in people with celiac disease. Celiac disease (also called celiac sprue, gluten intolerance, or gluten-sensitive enteropathy) is an autoimmune disorder characterized by intolerance to gluten. Gluten is a protein found in wheat, rye, and barley. It’s also sometimes found in oats that have been processed in plants that handle other grains.
According to the National Institutes of Health (NIH), 15 to 25 percent of people with celiac disease have DH. Celiac disease can also cause intense abdominal pain, constipation, nausea, and vomiting. People with DH typically don’t have any of the intestinal symptoms. However, even if they don’t experience any intestinal symptoms, 80 percent or more of people with DH still have intestinal damage, especially if they eat a diet that’s high in gluten, according to the National Foundation for Celiac Awareness (NFCA).
The intestinal damage and rash are due to the reaction of gluten proteins with a special kind of antibody called immunoglobulin A (IgA). Your body makes IgA antibodies to attack gluten proteins. When IgA antibodies attack gluten, they damage the parts of the intestines that allow you to absorb vitamins and nutrients. This sensitivity to gluten usually runs in families.
The structures formed when IgA attaches to gluten then enter the bloodstream, where they begin to clog small blood vessels, especially those in the skin. White blood cells are attracted to these clogs. The white blood cells release a chemical called “complement” that causes an itchy, blistery rash.
Who is at risk for dermatitis herpetiformis?
Celiac disease can affect anyone, but it tends to be more common in people who have another family member with celiac disease or DH.
Although more women than men are diagnosed with celiac disease, men are more likely to develop DH than women, according to the . The rash usually begins in your 20s or 30s, though it can start in childhood. The condition more commonly occurs in people of European descent. It less commonly affects people of African or Asian descent.
What are the symptoms of dermatitis herpetiformis?
DH is one of the itchiest rashes possible. Common locations of the rash include:
- lower back
- back of the neck
The rash is usually the same size and shape on both sides of the body and often comes and goes.
Before a full outbreak of the rash, you may feel the skin in a rash-prone area burn or itch. Bumps that look like pimples filled with clear liquid start to form. These are quickly scratched off. The bumps heal within a few days and leave a purple mark that lasts for weeks. But new bumps continue to form as old ones heal. This process can continue for years, or it can go into remission and then return.
While these symptoms are commonly associated with dermatitis herpetiformis, they can also be caused by other skin conditions such as atopic dermatitis, irritant or allergic contact dermatitis, psoriasis, pemphigoid, or scabies.
How is dermatitis herpetiformis diagnosed?
DH is best diagnosed with a skin biopsy. A doctor takes a small sample of skin and examines it under a microscope. Sometimes, a direct immunofluorescence test is done, in which the skin around the rash is stained with a dye that will show the presence of IgA antibody deposits. The skin biopsy can also help determine if the symptoms are caused by another skin condition.
Blood tests to check for these antibodies in the blood may also be done. An intestinal biopsy may be performed to confirm the presence of damage due to celiac disease.
If the diagnosis is uncertain, or another diagnosis is possible, other tests may be performed. Patch testing is the best way to diagnose allergic contact dermatitis, which is a common cause of symptoms similar to dermatitis herpetiformis.
If you don’t already have a dermatologist, you can browse doctors in your area through the Healthline FindCare tool.
What treatments are available for dermatitis herpetiformis?
DH can be treated with an antibiotic called dapsone. Dapsone is a powerful medicine with serious side effects. The dose must be increased slowly over several months before it’s fully effective.
Most people see relief from taking dapsone, but side effects may include:
Dapsone may also have negative interactions with other medications, such as aminobenzoate potassium, clofazimine, or trimethoprim.
Other drugs that may be used include tetracycline, sulfapyridine, and some immunosuppressive drugs. These are less effective than dapsone.
The most effective treatment that’s free of side effects is strict adherence to a gluten-free diet. This means you should completely avoid food, drink, or medicines containing the following:
Although this diet can be difficult to follow, it will have the most beneficial effect on your health if you have celiac disease. Any reduction in gluten intake may help lessen the amount of medication you will need to take.
What are the complications of dermatitis herpetiformis?
People with untreated DH and celiac disease may have a higher risk of intestinal cancer due to the constant inflammation in the intestines. Vitamin deficiencies and anemia may also be a problem if the intestines aren’t absorbing nutrients properly.
Since DH is an autoimmune disease, have found that it’s also associated with various other types of autoimmune diseases. These include:
What is the long-term outlook for dermatitis herpetiformis?
DH is a lifelong disease. You may go into remission, but any time you’re exposed to gluten, you may have an outbreak of the rash. Without treatment, DH and celiac disease can result in many negative health effects, including vitamin deficiencies, anemia, and gastrointestinal cancer.
Treatment with dapsone can control the rash symptoms rather quickly. However, the intestinal damage caused by celiac disease can only be treated by maintaining a strict gluten-free diet. Make sure to discuss any specific dietary considerations with your doctor or nutritionist.
What to know about dermatitis herpetiformis
Dermatitis herpetiformis is a skin rash that develops as a result of gluten sensitivity.
About of people with celiac disease also develop dermatitis herpetiformis. There is no cure for the condition, but people can reduce the symptoms with medicines and lifestyle changes.
The National Organization for Rare Disorders (NORD) consider dermatitis herpetiformis, or DH, a rare disease. It appears to affect people aged 30–40 years and rarely affects children. Men are at a slightly higher risk than women.
This article will discuss the symptoms, causes, and treatment of DH, and how it relates to celiac disease.
The symptoms of DH vary for different people, so not everyone may experience all symptoms. Typically, it affects three areas of the body — the skin, the gastrointestinal (GI) tract, and the mouth.
The most common effects of DH appear on the skin, with symptoms such as itchy blisters and raised clusters of lesions.
Commonly, areas that DH affects include elbows, knees, buttocks, and scalp. It may also affect the face and groin.
Many people feel an intense need to scratch itchy or burning areas.
The GI tract
Stomach inflammation and damage to the small intestine are common reactions in people with gluten sensitivity. These reactions usually develop a few days after a person ingests gluten.
People with DH may notice uncomfortable symptoms such as:
Some people develop problems with their tooth enamel, for example, horizontal grooves or discoloration.
Rarely, DH can also lead to oral ulcerations and canker sores.
Causes of DH
Although doctors still do not fully understand the causes of DH, genetics and lifestyle factors are likely to play a role.
For a long time, doctors did not know the cause of this skin rash, which made the condition challenging to manage. Experts then observed that DH symptoms reduced considerably in people who altered their diets to include little to no gluten.
This observation led to the discovery that DH has a strong association with gluten sensitivity. Gluten is a protein in grains that include wheat, rye, and barley.
Examples of foods that contain gluten are:
- baked goods
Some studies show that close relatives of people with DH are at an increased risk of developing the condition themselves.
To diagnose the cause of a skin rash, doctors will usually take a skin scraping or a skin biopsy. These tests involve a doctor obtaining a small sample of skin to send off to a laboratory for examination.
A doctor may also recommend an intestinal biopsy, especially in people who show signs of gastrointestinal problems.
When a skin biopsy cannot confirm or rule out DH, doctors may recommend blood tests that search for antibodies called anti-gliadin, anti-reticulin, and anti-endomysial antibodies.
A strict gluten-free diet is the most effective treatment option for people with DH. A registered dietician can help identify and eliminate both obvious and hidden sources of dietary gluten and recommend alternatives for the short- and long-term.
Doctors often prescribe a medicine called dapsone to provide immediate relief from symptoms.
Some people, however, cannot tolerate dapsone treatment. These individuals can instead take other forms of medication that contain substances called sulfapyridine or sulfamethoxypyridazine.
People may continue to take this medication for anywhere between . After this, DH can go into remission. Remission is when a person has no symptoms for more than 2 years while not using medication or a special diet.
DH and similar conditions
People may mistake DH for other conditions with similar symptoms, including:
- Eczema: Also known as atopic dermatitis, eczema is a group of skin conditions that cause skin inflammation or irritation. Common symptoms include dry skin, rashes, and itching, especially on the arms and behind the knees.
- Scabies: Scabies is a skin condition caused by a mite infestation. It is highly contagious and spreads by direct skin contact.
- Linear IgA disease: This disease is a rare and often long-term skin problem the characteristics of which are groups of itchy blisters and raised lesions on the skin. It is an autoimmune disease that certain medications can trigger.
DH is a long-term, chronic condition and tends to persist lifelong in most people.
People can control their symptoms with a combination of dapsone treatment and a gluten-free diet. After several years, the condition can go into remission.
People with DH can manage their condition by ensuring regular medical checkups and guidance from a specialist skin doctor, called a dermatologist, and a nutritionist.
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