A 40-year-old woman presented with facial edema and painful skin lesions involving the hands. On examination, heliotrope rash [Figure 1a], hyperkeratotic ulcerated papules on the knuckles [Figure 1b] and erythematous, flat-to-raised keratotic lesions involving the palmar aspect of the interphalangeal joints [Figure 1c] were noted. A diagnosis of dermatomyositis (DM) was made with clinical features and raised muscle enzymes.Figure 1: Clinical photograph showing heliotrope with periorbital edema (a) and Gottron’s sign over dorsal aspect of hand (b), flat-to-raised lesions involving palmar aspect of the fingers in a distribution “inverse” of the classic Gottron’s papules/sign (c, black arrow) and fingertip scars suggestive of healed ulcers (c, white arrow)Gottron’s papules are pathognomonic of DM. Inverse Gottron’s sign/papules [Figure 1c], due to vasculopathy are a rare but specific cutaneous finding in DM, usually associated with digital ulceration, interstitial lung disease, and anti-MDA5 autoantibodies.[1-3] Anti-MDA5-positive DM occurs in 7.2% of adults and 9.7% of children, respectively, in the Indian subcontinent. Cutaneous vasculopathic features such as cutaneous ulceration, and periorbital edema as seen in the index may indicate a worse poor prognosis in adults with anti-MDA-5 DM.[4] Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.
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