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:

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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