Penicillamine dermatopathy with lymphangiectases. A clinical, immunohistologic, and ultrastructural study.

Abstract

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.

• Authors: J B Goldstein, N S McNutt, G W Hambrick Jr, A Hsu
• Journal: Archives of dermatology (Arch Dermatol) Vol. 125 Issue 1 Pg. 92-7 (Jan 1989) ISSN: 0003-987X [Print] United States
• PMID: 2642686 (Publication Type: Case Reports, Journal Article)

Chemical References

• Collagen
• Elastin
• Penicillamine

Topics

• Adult
• Biopsy
• Collagen (biosynthesis)
• Connective Tissue (ultrastructure)
• Elastin (biosynthesis)
• Hepatolenticular Degeneration (drug therapy)
• Humans
• Immunoenzyme Techniques
• Lymphangiectasis (chemically induced, immunology, pathology)
• Male
• Microscopy, Electron (methods)
• Penicillamine (adverse effects, therapeutic use)
• Skin (immunology, ultrastructure)
• Skin Diseases (chemically induced, metabolism, pathology)