Dermatitis herpetiformis, also known as DH and Duhring’s disease, is a chronic skin condition caused by a reaction to gluten ingestion. The vast majority of patients with DH also have an associated gluten sensitive enteropathy (celiac disease).
Dermatitis herpetiformis (DH) is a relapsing cutaneous disease caused by gluten sensitivity and is characterized by severely pruritic papulovesicles or excoriated papules on the extensor surfaces, scalp, nuchal area, and buttocks. DH is considered an extraintestinal manifestation of celiac disease (CD).
Jan 27, 2016 · Dermatitis herpetiformis is a rare but persistent immunobullous disease that has been linked to coeliac disease (the American spelling is celiac), a gluten-sensitive enteropathy. The name herpetiformis is derived from the tendency for blisters to appear in clusters, resembling herpes simplex.
Dermatitis herpetiformis (DH) is an intensely itchy skin disease. It causes clusters of small blisters and small bumps. DH is caused by a sensitivity to gluten. The symptoms of DH may clear when all gluten is …
The vast majority of patients with DH also have an associated gluten sensitive enteropathy 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, and along the hairline. They are caused by gluten ingestion. However, DH patients with a normal intestinal biopsy and normal celiac serology blood test will still respond to a gluten-free diet. Symptoms tend to come and go, and DH is commonly diagnosed as eczema. Symptoms normally resolve with a strict, gluten-free diet. DH patients frequently 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 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? These IgA antibodies are directed against epidermal transglutaminase. The antibodies then travel to the skin where they bind with the epidermal transglutaminase protein. Gluten ingestion seems to trigger this reaction. A skin biopsy is used to confirm a diagnosis of DH. 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 immediately 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. 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. Skin lesions usually clear on the gluten-free diet. There are exceptions, however. Dapsone or sulfapyridine therapy may need to be continued for 1—2 years to prevent further DH outbreaks. In some cases, a diet high in iodine may worsen DH symptoms. If you are experiencing DH flare-ups, you should consult with a dermatologist expert in celiac disease to determine if foods or medicines high in iodine are the cause. Gluten-Free Gluten-Free Recipes. 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. Related Links Icon. Sign up for our mailing list.
DH is an external manifestation of an abnormal immune response to gluten, in which IgA antibodies form against the skin antigen epidermal transglutaminase. Their most common side effect is GI upset, but less frequently may cause hematologic toxicities neutropenia, agranulocytosis, thrombocytopenia, aplastic anemia in the first 1—3 months of starting the medication. Who is at risk for dermatitis herpetiformis? Dapsone is a well-tolerated medication in DH patients [ 69 ]. Consequently, patients with DH may have a history of GI symptoms such as bloating, diarrhea, or constipation, but these are usually minor if present. Enteropathy Most patients with DH have evidence of some degree of celiac-type damage in their small bowel; however, this is usually milder than CD. Fibrillar IgA deposition in dermatitis herpetiformis--an underreported pattern with potential clinical significance. Institutional Review Board Statement Not applicable. Dermatitis herpetiformis: From the genetics to the development of skin lesions. The gut may also have the same allergy to gluten. Related Links Icon. However, this assay has been commercially available in the US for almost a decade. Most patients with DH have evidence of some degree of celiac-type damage in their small bowel; however, this is usually milder than CD. Powell G. Skin biopsy is usually necessary to confirm dermatitis herpetiformis. Due to increased number of affected individuals with DH or CD among the family members of patients with DH, some authors advocate serologic testing for CD and DH among first- and second-degree family members [ 18 ]. Dermatitis herpetiformis Duhring with palmoplantar keratosis. In addition, as rituximab has been shown to be effective in a recalcitrant case of DH, other biologics may be of therapeutic use as well, but further investigation is needed. Strict adherence to gluten-free diet reduces this rare but serious long-term complication. However, our modern approach uses anti-TG3, instead of anti-TG2, antibody levels as the primary serological diagnostic marker, when available. This modification is the critical step that causes gliadin to have a stronger affinity for HLA DQ2 and DQ8 on antigen presenting cells. Other medications such as superpotent or potent topical steroids can also be considered for acute symptomatic relief and reduction of localized lesions in DH [ 33 ]. Dermatitis herpetiformis Duhring—Evaluation of disease severity and tissue transglutaminase levels in patients on dapsone therapy. Duhring Brocq disease, Duhring disease. Thyroid function tests are usually recommended due to the association between dermatitis herpetiformis and thyroid disease. Dermatitis herpetiformis and neurological dysfunction. Successful treatment of dermatitis herpetiformis with tetracycline and nicotinamide in a patient unable to tolerate dapsone. Because pruritus is such a prominent feature, its absence strongly favors another diagnosis [ 9 ]. Johnson H. Histology of lesional skin may show microabscesses containing neutrophils and eosinophils. How does a disorder that damages the intestines show up on the skin? The decreasing prevalence of severe villous atrophy in Dermatitis herpetiformis: A year experience in patients. These may also be used in combination with dapsone to achieve a more complete response [ 79 ]. Granular deposits of immunoglobulins in the skin of patients with dermatitis herpetiformis. A burning sensation may precede lesion formation. Fry L. The typical histopathological findings of DH are subepidermal vesicles and blisters with an accumulation of neutrophils at the tips of dermal papillae papillary microabscesses with relative sparing of the lower tips of rete ridges as seen in [ 53 ] Figure 2. Pruritic diseases such as urticaria, atopic dermatitis, eczema, scabies, prurigo, and lichen planus may be confused clinically with DH as well. Graziano M. Interleukin IL levels were found to be significantly elevated in DH as compared to other autoimmune bullous diseases [ 46 ]. DH is a chronic, remitting disease that is likely to be a lifelong condition in those who are diagnosed. However, the degree of small bowel damage at diagnosis does not affect the long-term prognosis of DH [ 56 ]. Patient and family communication should emphasize the importance of diet adherence even in the absence of GI manifestations, as well as the chronicity of the disease and its management. The pathogenesis of DH differs from CD as high-affinity anti-TG3 antibodies deposit in the dermal papillae and form a complex with TG3 produced by keratinocytes; this triggers a local inflammatory response within the papillary dermis that is predominantly neutrophilic. The primary drug used in DH treatment is dapsone, a sulfonamide that has both anti-inflammatory and antibacterial properties. Dermatitis herpetiformis effectively treated with heparin, tetracycline and nicotinamide.
Learn more. Dermatitis herpetiformis DH is an intensely itchy skin disease. It causes clusters of small blisters and bumps. It typically affects people in their 30s to 50s, but it can happen at any age. This lifelong condition affects more men than women. Despite its name, the herpes virus does not cause DH. DH is caused by a sensitivity or intolerance to gluten. Gluten is a protein found in wheat and grains. When you have DH and eat food with gluten, the gluten combines with an antibody from the intestines. As the gluten and antibody circulate in the blood, they clog small blood vessels in the skin. This is what causes the rash. DH is found most often in people of northern European heritage. The following diseases increase your risk of DH:. Celiac disease. Type 1 diabetes. The following are the most common symptoms of DH. However, each person may experience symptoms differently. Symptoms may include:. Clusters of itchy, small blisters and bumps, mostly on the elbows, lower back, buttocks, knees, and back of the head. The gut may also have the same allergy to gluten. This is known as celiac disease. You can have both DH and celiac. Some cases of celiac become cancerous. Because of this, if you have celiac disease, it is important to see a healthcare provider who specializes in the stomach and intestines a gastroenterologist. The symptoms of DH may look like other skin conditions. Always talk with your healthcare provider for a diagnosis. In addition to a medical history and physical exam, DH is usually confirmed with a skin biopsy and a specialized type of immunofluorescent stain that helps to detect the IgA antibodies. You may also have a blood tests to find certain antibodies. DH may be well-controlled with treatment. Specific treatment will be determined by your healthcare provider based on:. The symptoms of DH may go away if you cut all gluten from your diet. Healing may take several weeks to months. Your healthcare provider may also prescribe a medicine called dapsone. This medicine suppresses the skin response and may improve symptoms. However, the medicine has some side effects, including anemia. If dapsone is prescribed for you, your healthcare provider will carefully monitor your blood count. There is no known way to prevent this disease. You may be able to prevent complications by avoiding foods that contain gluten. Although difficult, sticking to a gluten-free diet can reduce the amount of medicines needed to manage the disease. People with DH often have celiac disease, which may develop into intestinal cancer. Thyroid disease may also develop. It causes clusters of small blisters and small bumps. Health Home Conditions and Diseases. What causes dermatitis herpetiformis? Who is at risk for dermatitis herpetiformis?