Increased fibroblast elastase activity in acquired cutis laxa.
BACKGROUND
Acquired cutis laxa is a rare disease characterized by sagging skin, premature wrinkling and reduced skin elasticity.


METHODS
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.


CONCLUSIONS
Our findings suggest that the inflammatory cells could play a significant role in the destruction of elastic fibres.
Bouloc
A
A
Department of Dermatology, Hôpital Henri-Mondor, Créteil, France. bouloc@univ-paris12.fr
Godeau
G
G
Zeller
J
J
Wechsler
J
J
Revuz
J
J
Cosnes
A
A
D000328:Adult; D001706:Biopsy; D003483:Cutis Laxa / Q000201:enzymology / Q000473:pathology*; D004547:Elastic Tissue / Q000201:enzymology / Q000473:pathology; D005260:Female; D005347:Fibroblasts / Q000166:cytology / Q000201:enzymology*; D006801:Humans; D010196:Pancreatic Elastase / Q000378:metabolism*; D012867:Skin / Q000473:pathology; D014581:Urticaria / Q000201:enzymology / Q000473:pathology
D010196:Pancreatic Elastase