Dermatitis Herpetiformis

Published on 23/06/2015 by admin

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Last modified 23/06/2015

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Chapter 160 Dermatitis Herpetiformis

image General Considerations

Dermatitis herpetiformis (DH) is a cutaneous manifestation of gluten-sensitive enteropathy (celiac disease). Patients with DH demonstrate serum IgA antibodies against epidermal transglutaminase and tissue transglutaminase.1 The putative autoantigen of DH is epidermal transglutaminase. DH has been referred to as “celiac disease of the skin.”2 Just as in celiac disease (CD), a gluten-free diet is most often all that is required to resolve the lesions. Absorption studies (see Chapter 20) can be used to assess the degree of enteropathy.

Most individuals with CD have celiac-associated antibodies and specific pairs of allelic variants in two HLA genes, HLA-DQA1 and HLA-DQB1.3 Only 3% of individuals with one or both of these alleles develops CD, yet 30% of the general population has one of them. Therefore, their presence is not diagnostic of CD but their absence excludes a diagnosis of CD. Genetic testing is available for the assessment of CD.

The average age at onset of DH is 7.2 years; it has a predilection for the elbows, knees, and buttocks. Skin biopsy shows granular or fibrillar IgA deposits.4 The characteristic skin lesions found in patients with DH are extremely itchy grouped vesicles most frequently located on extensor surfaces. Intense pruritus is the predominate symptom; however, DH is a clinical chameleon and can present with excoriations, eczematous lesions, or minimal patterns of discrete erythema or digital purpura.5