Dermatitis Herpetiformis (Duhring’s Disease)

Figure 3.28 A: Epidermolysis bullous a acquisita (EBA)* B: Dermatitis herpetiformis* C: Dermatitis herpetiformis, DIF* *Courtesy of Dr. Paul Getz

• Recurrent chronic pruritic disease associated with gluten-sensitive enteropathy
• Gluten: general name for storage proteins found in wheat, rye, and barley
  • NOT found in rice, oats or corn
  • Gliadin: soluble fraction; likely antigenic component
• Autoantigen: epidermal transglutaminase (TG-3), tissue transglutaminase (endomysial)
• Clinical: erythematous grouped papules or vesicles over elbows, knees, buttocks; intensely pruritic, so primary lesions typically not visible due to excoriations
• Histology: neutrophilic microabscesses in dermal papillae, ± subepidermal vesicles
• DIF: granular IgA > C3 deposition in dermal papillae
• IIF: negative
• Labs: anti-gliadin/anti-endomysial antibodies in DH/celiac disease
• Treatment: dapsone (immediate skin improvement), referral to GI (>90% with gluten-sensitive enteropathy and ↑ risk of small bowel lymphoma)
• ↑ Incidence thyroid disease (Hashimoto’s thyroiditis), IDDM, enteropathyassociated T cell ymphoma