Abnormalities of fibrillin in acquired cutis laxa.
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.
Lebwohl
M G
MG
Department of Dermatology, Mount Sinai School of Medicine, New York, NY.
Schwartz
E
E
Jacobs
L
L
Lebwohl
M
M
Sakai
L
L
Fleischmajer
R
R
D008841:Actin Cytoskeleton / Q000473:pathology; D000328:Adult; D003483:Cutis Laxa / Q000473:pathology*; D004547:Elastic Tissue / Q000473:pathology; D004549:Elastin / Q000032:analysis; D004817:Epidermis / Q000473:pathology; D005148:Facial Dermatoses / Q000473:pathology; D005260:Female; D000071837:Fibrillins; D005455:Fluorescent Antibody Technique; D006801:Humans; D008840:Microfilament Proteins / Q000032:analysis* / Q000172:deficiency; D016253:Microscopy, Immunoelectron
D000071837:Fibrillins; D008840:Microfilament Proteins; D004549:Elastin