Progressive dermopathy akin to pseudoxanthoma elasticum after D-penicillamine use in a patient with cystinuria

A 62-year-old woman with longstanding primary Sjogren’s Syndrome presented with wrinkling and paper-thin skin. First noticed on the nape, the condition progressed to her neck anteriorly (Fig. 1A), antecubital fossae laterally (Fig. 1B) and trunk caudally (Fig. 1C) over the years. She was diagnosed with hereditary cystinuria 23 years ago with recurrent nephrolithiasis and thereafter commenced on a 1000 mg daily dose of D-Penicillamine. Three years after treatment, she developed cutaneous features akin to pseudoxanthoma elasticum. Two of her siblings (also on d-penicillamine) reported similar findings without any background history of familial dermopathy. After dose reduction, some of these cutaneous changes reversed, albeit partially. Pseudoxanthoma elasticum is characterized by the progressive mineralization and disintegration of elastic fibers resulting in yellowish papules and plaques. An initial ‘plucked chicken skin’ appearance may suggest hyperlaxity of flexural surfaces and fragility of pressure zones. D-Penicillamine in high or prolonged doses can also disrupt collagen and elastin cross-linking by inhibiting the copper-dependent enzyme lysyl oxidase. Drug-induced pseudo-pseudoxanthoma elasticum, elastosis perforans serpinginosa, cutis laxa and anetoderma are thus reported in up to half of those on prolonged medication [1]. With the slow reversal of D-Pen-induced degenerative dermopathies and no medical treatment to expedite the resolution, drug replacement with trientine and/or zinc salts is recommended [2].