Dermatitis Herpetiformis: Causes, Symptoms, Diagnosis, Treatment, and Prevention
A chronic skin disease, Dermatitis Herpetiformis (DH), causes itchy, blistering, burning rashes in areas of the body such as elbows, knees, scalp, back, and buttocks. Duhring’s disease is also called gluten rash or Duhring’s disease. In DH, the skin and gut are both affected by an autoimmune reaction caused by gluten, a protein found in wheat, rye, barley, and oats. This is a manifestation of celiac disease, a condition that damages the lining of the small intestine and impairs nutrient absorption.
Causes and Risk Factors
The exact cause of DH is unknown, but it involves the production of antibodies called immunoglobulin A (IgA) that react with gluten. Inflammation and blistering result from these antibodies forming complexes in the small blood vessels of the skin, attracting white blood cells and attracting white blood cells. Celiac disease is caused by the same process in the intestine.
DH is more common in people who have a genetic predisposition to celiac disease, especially those who carry certain variants of the human leukocyte antigen (HLA) gene, such as HLA-DQ2 or HLA-DQ8. The prevalence of these genes is about 90% among individuals with DH, compared with about 40% of the general population. DH can affect anyone, but is more prevalent in Caucasians, men, and people of European descent. It usually occurs during adulthood, between the ages of 15 and 50, but it can also occur in children.
Some factors that may trigger or worsen DH include:
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Eating gluten-containing foods, such as bread, pasta, cereals, cakes, cookies, etc.
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Taking medications that contain iodine, such as contrast agents for imaging tests, or antibiotics, such as amiodarone or rifampin
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Having infections, such as herpes simplex, hepatitis B, or HIV
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Having stress, trauma, or hormonal changes
Symptoms and Complications
Itchy, burning, and stingy rashes containing small blisters and bumps are the main symptom of DH. Typically, the rash appears symmetrically on both sides of the body, often affecting elbows, knees, scalps, backs, and buttocks. However, it can also occur on other areas, such as the face, neck, chest, abdomen, groin, and mouth. The rash may vary in severity and duration, and it may come and go over time. Scratching the rash can result in bleeding, scarring, and infection.
DH may also cause other symptoms related to celiac disease, such as:
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Abdominal pain, bloating, gas, diarrhea, or constipation
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Weight loss, fatigue, anemia, or malnutrition
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Osteoporosis, arthritis, or joint pain
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Infertility, miscarriage, or menstrual problems
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Headaches, depression, anxiety, or nerve damage
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Mouth ulcers, dental enamel defects, or dry mouth
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Skin pigmentation changes, such as darkening or lightening of the skin
DH can lead to serious complications if left untreated, such as:
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Skin infections, such as impetigo, cellulitis, or sepsis
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Intestinal cancer, such as lymphoma or adenocarcinoma
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Other autoimmune diseases, such as type 1 diabetes, thyroid disease, or Addison’s disease
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Vitamin and mineral deficiencies, such as iron, folate, calcium, vitamin D, or vitamin B12
Diagnosis and Treatment
Doctors who specialize in skin diseases and gastroenterologists who specialize in digestive diseases can diagnose DH using the following tests:
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Skin biopsy: A small sample of skin is taken from the affected area and examined under a microscope for the presence of IgA deposits and inflammation.
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Blood test: A blood sample is taken and tested for the presence of IgA antibodies against gluten and other proteins, such as transglutaminase and endomysium.
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Intestinal biopsy: A small sample of the lining of the small intestine is taken through an endoscope, a thin tube inserted through the mouth or nose, and examined under a microscope for signs of damage and inflammation.
The treatment of DH aims to relieve the symptoms and prevent the complications. The treatment includes:
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DH is treated with the antibiotic dapsone, which reduces inflammation and itching. The side effects of dapsone, however, include anemia, liver problems, or allergic reactions, so regular blood tests are required to monitor its safety. Aside from antibiotics, there are also anti-inflammatory drugs, such as corticosteroids, that may be used to treat DH, including sulfapyridine, sulfasalazine, and tetracycline. Medications can be taken orally or applied topically.
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A gluten-free diet is the best way to treat DH. All foods and products containing wheat, rye, barley, and some oats should be eliminated, as well as checking labels for hidden gluten in processed foods, medications, supplements, and cosmetics. It is possible to reduce antibodies, heal the intestine, and prevent recurrence of the rash with a gluten-free diet. It may take several months or years for the rash to clear entirely, depending on the severity and duration of the disease. Gluten-free diets can also be used to prevent or reduce celiac disease symptoms and complications. Dietitians, health professionals who specialize in nutrition, can assist patients in planning a gluten-free diet that meets their nutritional needs.
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Other measures: Some other measures that can help manage DH include:
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Applying moisturizers, emollients, or soothing creams to the skin to reduce dryness and irritation
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Taking antihistamines, such as diphenhydramine or cetirizine, to reduce itching and scratching
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Wearing loose-fitting, cotton clothing to avoid friction and pressure on the skin
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Avoiding triggers, such as iodine, infections, stress, or trauma, that may worsen the rash
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Seeking support from family, friends, or support groups to cope with the emotional and social impact of the disease
Prevention and Outlook
As DH is an autoimmune and genetic disease, there is no known way to prevent it. However, following a gluten-free diet can help reduce flare-ups and complications. In addition to monitoring their skin and intestinal health, people with DH should also visit their doctors regularly to be screened for cancer and other diseases. In addition, people with DH should inform their family members of their condition, as they may be at risk of developing DH or celiac disease themselves.
In most cases, DH is a chronic, lifelong disease that can cause significant discomfort and distress. However, with the right diagnosis, treatment, and adherence to a gluten-free diet, most people with DH can achieve remission of their symptoms and improve their quality of life.
