Abnormalities of fibrillin in acquired cutis laxa.

Journal: Journal Of The American Academy Of Dermatology
Published:
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.

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

Authors
M Lebwohl, E Schwartz, L Jacobs, M Lebwohl, L Sakai, R Fleischmajer
Relevant Conditions

Cutis Laxa