Penicillamine dermatopathy with lymphangiectases. A clinical, immunohistologic, and ultrastructural study.
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.
Goldstein
J B
JB
Department of Medicine, New York Hospital-Cornell University Medical College, NY 10021.
McNutt
N S
NS
Hambrick
G W
GW
Hsu
A
A
D000328:Adult; D001706:Biopsy; D003094:Collagen / Q000096:biosynthesis; D003238:Connective Tissue / Q000648:ultrastructure; D004549:Elastin / Q000096:biosynthesis; D006527:Hepatolenticular Degeneration / Q000188:drug therapy*; D006801:Humans; D007124:Immunoenzyme Techniques; D008200:Lymphangiectasis / Q000139:chemically induced* / Q000276:immunology / Q000473:pathology; D008297:Male; D008854:Microscopy, Electron / Q000379:methods; D010396:Penicillamine / Q000009:adverse effects* / Q000627:therapeutic use; D012867:Skin / Q000276:immunology / Q000648:ultrastructure; D012871:Skin Diseases / Q000139:chemically induced* / Q000378:metabolism / Q000473:pathology
D003094:Collagen; D004549:Elastin; D010396:Penicillamine