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D‐Penicillamine Treatment for Lipoid Proteinosis
Author(s) -
Kaya Tamer Irfan,
Kokturk Aysin,
Tursen Umit,
Ikizoglu Guliz,
Polat Ayse
Publication year - 2002
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1046/j.1525-1470.2002.00104.x
Subject(s) - penicillamine , medicine , hyaline , elastin , cystinuria , pathogenesis , pathology , aminoaciduria , fibroblast , cystinosis , cystine , cell culture , biology , biochemistry , urine , cysteine , enzyme , genetics
Lipoid proteinosis, a rare disorder inherited in an autosomal recessive fashion, is characterized by the deposition of hyaline‐like material in the skin, mucous membranes, and other tissues. Perturbation of collagen metabolism has been suggested to play an important role in the pathogenesis. No effective therapy is available for the disease. The chelating agent D‐penicillamine has long been used to treat several diseases. In addition to its immunosuppressive and anti‐inflammatory effects, it also impairs fibroblast proliferation and inhibits the formation of the cross‐links in collagen and elastin fibers. A 13‐year‐old girl was clinically and histologically diagnosed with lipoid proteinosis. We treated her with 600 mg/day of D‐penicillamine for 2 years. The patient had improved clinically and histopathologically by the end of this treatment. We suggest D‐penicillamine as a promising agent, even in low doses, for the treatment of lipoid proteinosis.