Dermatitis herpetiformis

DERMATITIS HERPETIFORMIS
Signs and Symptoms:
  • Intensely itchy vesicles, papulovesicles and urticaria papules symmetrically distributed over extensor surfaces (elbows, knees, buttocks, back, scalp, and posterior neck) Almost all have an associated, usually subclinical, gluten-sensitive enteropathy
Pathophysiology and Diagnosis:
  • >90% express the HLA-B8/DRw3 and HLA-DQw2 haplotypes
  • Biopsy of new lesions - vesicle forms subepidermally and develops from collections of inflammatory cells in the papillary tips (the papillary tip abscess)
  • Direct IF- presence of IgA in the papillary tips in the skin around the lesions
Treatment:
  • Gluten-free diet
  • Dapsone therapy
  • Patients are at increased risk for development of gastrointestinal lymphoma
Exam Question
  • Intensely itchy vesicles, papulovesicles and urticaria papules symmetrically distributed over extensor surfaces (elbows, knees, buttocks, back, scalp, and posterior neck) Almost all have an associated, usually subclinical, gluten-sensitive enteropathy
  • >90% express the HLA-B8/DRw3 and HLA-DQw2 haplotypes
  • Biopsy of new lesions - vesicle forms subepidermally and develops from collections of inflammatory cells in the papillary tips (the papillary tip abscess)
  • Direct IF- presence of IgA in the papillary tips in the skin around the lesions
  • Gluten-free diet
  • Dapsone therapy
  • Patients are at increased risk for development of gastrointestinal lymphoma

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