Dermatitis Herpetiformis

Dermatitis Herpetiformis

 

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

Epidemiology

  • Incidence – 10-39 per 100,000
  • Age of onset – childhood to older years; peak onset 20-40 year olds
  • Gender – M:F, almost equal with slight male predominance
  • Ethnic – most common in north European descendants, occurs in all ethnic groups

Risk Factors

  • Gluten-sensitive enteropathy
  • Lymphoma
  • Autoimmune diseases including lupus, thyroid, diabetes
  • Oral aphthae

Pathology and Immunopathology

  • Granular and/or fibrillar deposition of IgA 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 located on extensor surfaces (elbows and knees) in a symmetrical distribution
  • Chronic eczematoid skin changes

Diagnosis

  • Laboratory testing
    • IgA endomysial and tissue transglutaminase antibody testing
    • If IgA deficient and/or in atypical cases, IgG endomysial and tissue transglutaminase antibody testing
  • Biopsy
    • Skin biopsy
      • Perilesional
      • Additional perilesional biopsy increases possibility of detecting sparse IgA deposits

Differential Diagnosis

  • Contact dermatitis
  • Bullous impetigo
  • Pemphigoid
  • Pemphigus
  • Bullous lupus erythematosus

Treatment

  • Dietary modification to eliminate gluten ingestion
  • Dapsone

See Also