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 Important
- 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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