Dermatitis herpetiformis, also known as DH and Duhring’s disease, is a skin manifestation of celiac disease. Extremely itchy bumps or blisters appear on both sides of the body, most often on the forearms near the elbows, as well as on knees and buttocks.
What is Dermatitis Herpetiformis?
Dermatitis Herpetiformis bumps and blisters resemble herpes lesions, hence the name “herpetiformis”, but are NOT caused by the herpes virus. Symptoms of DH tend to come and go, and it is commonly diagnosed as eczema. Symptoms normally resolve when on a strict, gluten-free diet.
DH affects 15 to 25 percent of people with celiac disease who typically have no digestive symptoms. DH can affect people of all ages, but most often appears for the first time in those between the ages of 30 and 40. People of northern European descent are more likely than those of African or Asian heritage to develop DH. The condition is somewhat more common in men than women. And men are more likely to have atypical oral or genital lesions.
How does a disorder that damages the intestines show up on the skin? “When a person with celiac disease consumes gluten, the mucosal immune system in the intestine responds by producing a type of antibody called immunoglobulin A (IgA)”, explains John Zone, M.D., CDF Medical Advisory Board Member and Chairman of the Department of Dermatology at the University of Utah School of Medicine. As IgA enters the bloodstream, it can collect in small blood vessels under the skin, triggering further immune reactions that result in the blistering rash of DH.
Diagnosing DH With Skin Biopsy and Blood Tests
A skin biopsy is used to confirm a diagnosis of DH. Dermatologists usually use what’s called a “punch biopsy” to remove the skin and test it for dermatitis herpetiformis. After injecting a local anesthetic, your dermatologist will use a tiny, cookie-cutter-like punch to remove a 4mm sample of skin. The incision can be closed with one stitch and generally heals with very little scarring.
A skin sample is taken from the area next to a lesion and a fluorescent dye is used to look for the presence of Immunoglobulin A (IgA) deposits that appear in a granular pattern. Skin biopsies of people with DH are almost always positive for this granular IgA pattern.
It is important to have your dermatitis herpetiformis skin biopsy performed by someone who has diagnosed the skin condition before and knows how to do the biopsy. The skin sample must be taken from skin directly adjacent to the suspected dermatitis herpetiformis lesion, as opposed to directly from the lesion, since inflammation in the lesion can destroy the IgA deposits.
Blood tests for other antibodies commonly found in people with celiac disease—antiendomysial and anti-tissue transglutaminase antibodies—supplement the diagnostic process. If the antibody tests are positive and the skin biopsy has the typical findings of DH, patients do not need an intestinal biopsy to confirm the diagnosis of celiac disease.
Treatment for DH With Dapsone and the Gluten-Free Diet
If you are diagnosed with dermatitis herpetiformis, your dermatologist may prescribe dapsone for short-term relief from the itching. According to Dr. Zone, the rash responds dramatically to dapsone, usually in 48 to 72 hours. People who can’t tolerate dapsone may be given sulfapyridine or sulfamethoxypyridazine instead, although these drugs are less effective.
However, you’ll still need to follow a strict gluten-free diet to control your dermatitis herpetiformis. Even with a gluten-free diet, dapsone or sulfapyridine therapy may need to be continued for 1–2 years to prevent further DH outbreaks.