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Celiac Disease and Dermatitis Herpetiformis

Gluten sensitivity is relatively associated with several skin alterations but the most common presentation is in a form of chronic pruritic disease characterized by the presence of several symmetrical papulo-vesicular rash that evolves to crusting lesions broadly distributed over the body, but especially on the forearms, knees, buttocks, wrists and scalp. It is well known as dermatitis herpetiformis (DH) and the skin biopsy shows the characteristic lineal, granular deposits of IgA in the dermal papillae.

This condition affects about 15%-25% of patients with celiac disease and its presence is known as the “card presentation” of some celiac disease patients[31]. A gluten-free diet is the treatment of choice, although at the start of the diet, drug therapy may be added, usually dapsone, to effectively and quickly resolve the itching and the rash.

This drug suppresses the inflammation of the skin but obviously has no any infl uence on the intestinal abnormalities. The resolution of the cutaneous lesions may be slow, and some patients must wait 1-2 years after starting on a GFD, for the complete disappearance of DH.

The intestinal biopsies show identical changes to CD, but predominate with mild lesions and a patchy distribution. tTG positivity is also similar, although at lower levels, possible refl ecting a milder enteropathy. One study has shown the presence of antibodies exclusively against tTG-3 (also known as epidermal transglutaminase), a cytosolic enzyme involved in cell envelope formation during keratinocyte differentiation.

Although these findings remain to be confirmed, these may offer some clues to understanding the difference in clinical presentation between celiac disease patients with, or without, associated skin lesions.

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