Abnormalities of fibrillin in acquired cutis laxa

Mark G. Lebwohl; Elaine Schwartz; Lloydstone Jacobs; Raul Fleischmajer; Lynn Sakai

doi:10.1016/S0190-9622(94)70115-6

Abnormalities of fibrillin in acquired cutis laxa

Mark G. Lebwohl, Elaine Schwartz, Lloydstone Jacobs, Raul Fleischmajer, Lynn Sakai

Research output: Contribution to journal › Article › peer-review

18 Scopus citations

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 immunoelec-tron 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.

Original language	English (US)
Pages (from-to)	950-954
Number of pages	5
Journal	Journal of the American Academy of Dermatology
Volume	30
Issue number	6
DOIs	https://doi.org/10.1016/S0190-9622(94)70115-6
State	Published - Jan 1 1994
Externally published	Yes

ASJC Scopus subject areas

Dermatology

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10.1016/S0190-9622(94)70115-6

Cite this

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title = "Abnormalities of fibrillin in acquired cutis laxa",

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 immunoelec-tron 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.",

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T1 - Abnormalities of fibrillin in acquired cutis laxa

AU - Lebwohl, Mark G.

AU - Schwartz, Elaine

AU - Jacobs, Lloydstone

AU - Fleischmajer, Raul

AU - Sakai, Lynn

PY - 1994/1/1

Y1 - 1994/1/1

N2 - 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 immunoelec-tron 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.

AB - 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 immunoelec-tron 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.

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Abnormalities of fibrillin in acquired cutis laxa

Abstract

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