elastin and Lymphangiectasis

The term penicillamine dermatopathy refers to the characteristic hemorrhagic skin lesions found in persons receiving long-term penicillamine therapy for either Wilson's disease or cystinuria. These lesions are thought to develop as a result of faulty collagen and elastin synthesis. We describe a patient with Wilson's disease who developed extensive penicillamine dermatopathy. In addition, histologic, immunochemical, and ultrastructural studies revealed multiple lymphangiectases with blood vessel to lymphatic anastomosis within these lesions, a finding not previously reported. The possible relationship to defective collagen and elastin formation are considered.

Topics: Adult; Biopsy; Collagen; Connective Tissue; Elastin; Hepatolenticular Degeneration; Humans; Immunoenzyme Techniques; Lymphangiectasis; Male; Microscopy, Electron; Penicillamine; Skin; Skin Diseases