Abstract

Dermatomyositis is a type of idiopathic inflammatory myopathy (IIM), characterized by an autoimmune response primarily against skin and muscle tissues.1,2 A variety of cutaneous manifestations can be seen in dermatomyositis, although Gottron’s papules, Gottron’s sign, and a heliotrope rash are considered pathognomonic.1,3 Gottron’s papules are flat-topped, red-to-purple papules and plaques involving the skin, metacarpophalangeal, and interphalangeal joints of the dorsal aspect of both hands. These lesions resolve, leaving areas of dyspigmentation, atrophy, and scarring. Gottron’s papules should be differentiated from similar lesions, such as verruca vulgaris, lichen planus, knuckle pads, sarcoidosis, and erythema elevatum diutinum.1,3 If a clinician initially suspects a Gottron’s mimicker and treats with the standard treatment for that diagnosis (i.e., verruca without resolution), a biopsy should be considered. Morphea, dermatomyositis, celiac disease, and dermatitis herpetiformis are autoimmune disorders that share similar pathogenesis and triggers. The diagnosis of multiple autoimmune syndrome (MAS) is given to patients with at least three autoimmune disorders with involvement of one or more autoimmune endocrine disorders.4 We present a case describing a patient with morphea, dermatomyositis, and celiac disease/dermatitis herpetiformis, suggesting a constellation of autoimmune disorders not subclassified by the MAS classification and not reported in the literature.

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