Increased fibroblast elastase activity in acquired cutis laxa

Dermatology. 1999;198(4):346-50. doi: 10.1159/000018146.

Abstract

Background: Acquired cutis laxa is a rare disease characterized by sagging skin, premature wrinkling and reduced skin elasticity.

Observation: We report a 21-year-old woman, who presented with acquired cutis laxa on the face and the ear lobes. Urticarial papules had preceded for 6 years. There was no systemic involvement. Skin specimens were obtained from lax skin and urticarial papules, and from healthy controls. Histology showed only few perivascular lymphocytes in lax ear skin and a dense inflammatory infiltrate in urticarial skin. In both biopsies elastic fibres were decreased as demonstrated by computerized morphometric analyses. Elastase activities of fibroblasts in culture were evaluated. There was a 2- to 3-fold increase in elastase activity in urticarial skin fibroblasts, contrasting with a normal elastase activity in lax ear skin.

Conclusion: Our findings suggest that the inflammatory cells could play a significant role in the destruction of elastic fibres.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Biopsy
  • Cutis Laxa / enzymology
  • Cutis Laxa / pathology*
  • Elastic Tissue / enzymology
  • Elastic Tissue / pathology
  • Female
  • Fibroblasts / cytology
  • Fibroblasts / enzymology*
  • Humans
  • Pancreatic Elastase / metabolism*
  • Skin / pathology
  • Urticaria / enzymology
  • Urticaria / pathology

Substances

  • Pancreatic Elastase