Abnormalities of fibrillin in acquired cutis laxa

J Am Acad Dermatol. 1994 Jun;30(6):950-4. doi: 10.1016/s0190-9622(94)70115-6.

Abstract

Background: Degeneration of elastic tissue in acquired cutis laxa has been previously described, but microfibrils have not been adequately studied.

Objective: We determined whether the microfibrillar component of elastic tissue is affected in skin of a patient with acquired cutis laxa.

Methods: Lesional skin was examined with indirect immunofluorescence and immunoelectron microscopy with antibodies to fibrillin.

Results: Indirect immunofluorescence showed a reduction in the distribution of fibrillin in the papillary dermis, where there was loss of the usual pattern of microfibrils perpendicular to the epidermis. Immunoelectron microscopy showed a typical distribution of elastic microfibrils around elastin of normal skin. In skin affected by cutis laxa microfibrils appeared morphologically normal but appeared less frequently in selected sites.

Conclusion: The microfibrillar component of elastic fibers was reduced in the papillary dermis of this patient with acquired cutis laxa.

Publication types

  • Case Reports

MeSH terms

  • Actin Cytoskeleton / pathology
  • Adult
  • Cutis Laxa / pathology*
  • Elastic Tissue / pathology
  • Elastin / analysis
  • Epidermis / pathology
  • Facial Dermatoses / pathology
  • Female
  • Fibrillins
  • Fluorescent Antibody Technique
  • Humans
  • Microfilament Proteins / analysis*
  • Microfilament Proteins / deficiency
  • Microscopy, Immunoelectron

Substances

  • Fibrillins
  • Microfilament Proteins
  • Elastin