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Dermatitis Herpetiformis

  • Dermatitis Herpetiformis is a highly pruritic vesicular disease with characteristic morphology that is often encountered in the context of celiac disease. Onset is typically in the early adult, second through third, decades.
  • The pathogenesis of dermatitis herpetiformis is unclear but given its strong association with celiac disease may involve the deposition of IgA within the epidermis.
Morphology and Clinical Consequences
  • Disease is characterized by highly pruritic, small, clustered vesicles on an erythematous base usually in a symmetric distribution on the extensor surfaces, upper back, and sacrum. Because of its clustered vesicular gross morphology, the disease can clinically mimic herpetic eruption. Because of the intense pruritis, vesicles may not be seen clinically, replaced with excoriations secondary to scratching.
  • Histologically, blisters are subepidermal and begin at the tips of dermal papillae which later coalesce to form the grossly visible vesicles. Tips of dermal papillae also show a neutrophilic infiltrate and on immunofluorescence IgA deposition can be demonstrated in the same location.
  • Avoidance of gluten in the diet typically resolves both skin lesions and the enteropathy associated with celiac disease.