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For further information, see CMDT Part 6-24: Dermatitis Herpetiformis

Key Features

  • Highest prevalence in northern Europe

  • Is associated with HLA antigens -B8, -DR3, and -DQ2

  • Patients have gluten-sensitive enteropathy, but nondermatologic disease is subclinical in the great majority

  • Ingestion of gluten plays a role in the exacerbation of skin lesions

Clinical Findings

  • Uncommon disease manifested by pruritic papules, vesicles, and papulovesicles mainly on the elbows, knees, buttocks, posterior neck, scalp

  • Patients with dermatitis herpetiformis are at increased risk for gastrointestinal lymphoma, and this risk is reduced by a gluten-free diet

Diagnosis

  • Diagnosis is made by light microscopy, which demonstrates neutrophils at the dermal papillary tips

  • Direct immunofluorescence studies show granular deposits of IgA along the dermal papillae

  • Circulating antibodies to tissue transglutaminase are present in 90% of cases

  • Nonsteroidal anti-inflammatory drugs may cause flares

  • Differential diagnosis

    • Weeping or encrusted lesions (crusted lesions)

    • Impetigo

    • Contact dermatitis (acute)

  • Any vesicular dermatitis can become crusted

Treatment

  • Strict long-term avoidance of dietary gluten may

    • Eliminate the need for treatment

    • Decrease the dose of dapsone (usually 100–200 mg orally daily) required to control the disease

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