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

Arch Dermatol. 1989 Jan;125(1):92-7.

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.

Publication types

  • Case Reports

MeSH terms

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

Substances

  • Collagen
  • Elastin
  • Penicillamine