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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

  • Associated with gluten-sensitive enteropathy, but in the great majority it is subclinical

  • 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 has been shown to decrease the dose of dapsone (usually 100–200 mg orally daily) required to control the disease and may even eliminate the need for treatment

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