Systemic sclerosis-like lesions during long-term penicillamine therapy for Wilson's disease

Abstract

Systemic sclerosis-like lesions developed in a 14-year-old boy with Wilson's disease who had been treated with D-penicillamine for 11 years. Clinical and laboratory manifestations included proximal scleroderma, pulmonary restrictive defects, positive antinuclear antibodies, and the deposition of C3 at the dermal-epidermal junction of the lesional skin. This is the first case reported in which long-term administration of penicillamine was followed by the development of systemic sclerosis-like lesions.