An iatrogenic dermatosis with ulceration.

Abstract

Conflict of interest: none declared. A 36‐year‐old woman presented with a 1‐year history of denuded nodules on the perianal area. She complained of skin laxity in the axilla, groin and abdomen of 7 years' duration, and she had multiple, yellowish, 2–3 mm sized papules on her neck, giving a ‘plucked chicken’ appearance (Fig. 1). She had been on long‐term penicillamine therapy at a dose of 1 g daily since the age of 10 years for treatment of Wilson's disease. Skin examination demonstrated several denuded nodules with marginal induration on the perianal area (Fig. 2). ... ... Skin biopsy taken from lesions on the perianal area showed transepidermal elimination of the abnormal elastic fibres, which were thickened with prominent lateral protrusions, giving the so‐called ‘lumpy‐bumpy’ or ‘bramble‐bush’ appearance (Fig. 3). ... What is your diagnosis? Penicillamine‐induced degenerative dermatosis. Penicillamine is a copper chelator used at high doses for the treatment of Wilson's disease. An acute hypersensitivity eruption may appear within the first 10 days of treatment, and immunological disorders such as pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, systemic lupus erythematosus and dermatomyositis are well‐known manifestations that occur later in the course of therapy.1, 2 At higher doses of penicillamine, a spectrum of cutaneous elastotic and collagen disorders, including pseudo‐pseudoxanthoma elasticum, elastosis perforans serpiginosa, cutis laxa and anetoderma, can occur, and combinations of these have been also reported.1–3 Iozumi et al.3 proposed that these dermatoses should be summarized as one entity, penicillamine‐induced degenerative dermatoses, because of the pathogenic background and clinical similarities. The underlying mechanism is thought to involve direct inhibition of cross‐linkage of collagen fibres by penicillamine.4 In addition, the enzyme lysyl oxidase, required for cross‐linking of collagen fibres, is dependent on the presence of copper and therefore may be inhibited by removal of copper from the tissues by penicillamine.,1, 5