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The treatment of linear morphoea with D‐penicillamine
Author(s) -
CURLEY R. K.,
MACFARLANE A. W.,
EVANS S.,
WOODROW J. C.
Publication year - 1987
Publication title -
clinical and experimental dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.587
H-Index - 78
eISSN - 1365-2230
pISSN - 0307-6938
DOI - 10.1111/j.1365-2230.1987.tb01859.x
Subject(s) - penicillamine , medicine , disfigurement , scleroderma (fungus) , muscle contracture , deformity , localized scleroderma , dermatology , surgery , pathology , fibrosis , inoculation
Summary 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 demonstrated 2 , and the discovery that D‐penicillamine could normalize this ratio led Moynahan 3 to treat 14 children with linear morphoea with low‐dose D‐penicillamine. He reported uniformly good results in all patients. Kesler and colleagues 4 , 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.