The treatment of linear morphoea with D-penicillamine

Localized morphoea (localized scleroderma) generally has a good prognosis and only rarely evolves into systemic sclerosis. The linear form, however, can cause considerable disfigurement and disability due to the involvement of skin, subcutaneous tissue, muscle, bone, and synovium. When it occurs in childhood, growth arrest, limb shortening, and joint contractures may result in severe local deformity and rarely even loss of a limb.1 A lowered ratio of soluble to insoluble collagen in skin biopsies from patients with scleroderma has been demonstrated2, and the discovery that D-penicillamine could normalize this ratio led Moynahan3 to treat 14 children with linear morphoea with low-dose D-penicillamine. He reported uniformly good results in all patients. Kesler and colleagues4, however, found no benefit when comparing two children with linear morphoea, one of whom was treated with D-penicillamine. We report two patients with linear morphoea who showed striking improvement following treatment with 250 mg D-penicillamine daily.