Dermatitis Herpetiformis

 

Clinical Background

Dermatitis herpetiformis is a chronic, pruritic skin disease that is usually associated with intestinal gluten sensitivity (celiac disease).

Epidemiology

  • Incidence – 10-39/100,000
  • Age – all ages; peak onset is 20s-30s
  • Sex – M>F (minimal)
  • Ethnicity – most common in those of northern European descent but occurs in all ethnic groups

Risk Factors

  • Gluten-sensitive enteropathy (celiac disease)
  • Lymphoma
  • Autoimmune disease (eg, lupus, thyroid, diabetes)
  • Oral aphthae

Pathophysiology and Immunopathophysiology

  • Granular and/or fibrillar deposition of IgA antibodies in dermal papillae and, less commonly, in blood vessels
  • Neutrophilic microabscesses in the dermal papillae
  • Dermal infiltration of neutrophils and eosinophils

Clinical Presentation

  • Intense pruritus with excoriations
  • Papulovesicular lesions and urticarial wheals in a symmetrical distribution – classically involves extensor surfaces of arms, knees and buttocks
  • Chronic eczematoid skin changes

Treatment

  • Dietary modification to eliminate gluten ingestion
  • Dapsone