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Dermatitis herpetiformis is an intensely pruritic, chronic, autoimmune, papulovesicular cutaneous eruption in patients who have celiac disease. Typical findings are clusters of intensely pruritic, erythematous, urticarial lesions, as well as vesicles, papules, and bullae, usually distributed symmetrically on extensor surfaces. Diagnosis is by skin biopsy with direct immunofluorescence testing Treatment is usually with dapsone or sulfapyridine and a gluten-free diet.
Dermatitis herpetiformis often occurs in young adults but can occur in children and the elderly. It is rare in blacks and Asians.
All patients with dermatitis herpetiformis have celiac disease, but most are asymptomatic. Dermatitis herpetiformis develops in 15 to 25% of patients with celiac disease. Patients may have a higher incidence of other autoimmune disorders (including thyroid disorders, pernicious anemia, and diabetes) and small-bowel lymphoma. IgA deposits collect in the dermal papillary tips and attract neutrophils; they can be eliminated by a gluten-free diet.
The term herpetiformis refers to the clustered appearance of the lesions and does not indicate a relationship to herpesvirus.
Onset can be acute or gradual. Vesicles, papules, and urticarial lesions are usually distributed symmetrically on extensor aspects of the elbows, knees, sacrum, buttocks, and occiput. Lesions itch and burn. Because itching is intense and skin is fragile, vesicles tend to rupture quickly, often making intact vesicles difficult to detect. Oral lesions may develop but are usually asymptomatic. Iodides and iodine-containing preparations may exacerbate the cutaneous symptoms.
Diagnosis is based on skin biopsy and direct immunofluorescence testing of a lesion and adjacent (perilesional) normal-appearing skin. Granular IgA deposition in the dermal papillary tips is invariably present and important for diagnosis. All patients with dermatitis herpetiformis should be evaluated for celiac disease.
Dapsone generally results in remarkable improvement. Initial dosages of dapsone are 25 to 50 mg po once/day in adults and 0.5 mg/kg in children. Usually, this dose dramatically relieves symptoms, including itching and burning, within 1 to 3 days. If improvement occurs, the dose is continued. If no improvement occurs, the dose can be increased every week, up to 300 mg/day. Most patients respond well to 50 to 150 mg/day. Dapsone can cause hemolytic anemia; risk is highest after 1 mo of treatment and is increased in patients who have G6PD deficiency. Methemoglobinemia is common; hepatitis, agranulocytosis, dapsone syndrome (hepatitis and lymphadenopathy), and a motor neuropathy are more serious complications. Sulfapyridine 500 mg po tid (or, alternatively, sulfasalazine) is an alternative for patients who cannot tolerate dapsone. Doses of sulfapyridine up to 2000 mg po tid can be used. Sulfapyridine may cause agranulocytosis. Patients receiving dapsone or sulfapyridine should have a baseline CBC; CBC is then done weekly for 4 wk, then every 2 to 3 wk for 8 wk, and every 12 to 16 wk thereafter. Patients suspected of having G6PD deficiency should be tested for this deficiency before being treated with dapsone. If patients cannot tolerate dapsone or sulfonamides, heparin may be used alone or in combination with tetracycline and nicotinamide.
Patients are also placed on a strict gluten-free diet. After initial therapy and disease stabilization, most patients can stop drug therapy and be maintained on the gluten-free diet, but this may take months or years. A gluten-free diet also maximizes improvement in the enteropathy and, if strictly followed for 5 to 10 yr, decreases risk of lymphoma.
Patients who have dermatitis herpetiformis, even if they have no GI symptoms, have celiac disease and are at risk of small-bowel lymphoma.
Because itching is intense and skin is fragile, vesicles may all be broken and thus not evident on examination.
Confirm the diagnosis with skin biopsy and direct immunofluorescence testing of a lesion and adjacent normal-appearing skin.
Use dapsone or an alternative drug (eg, sulfapyridine) to control skin manifestations initially.
Have patients try to maintain long-term control with only a strict gluten-free diet so that drug therapy can be stopped.
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