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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)

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. CONCLUSION: The microfibrillar component of elastic fibers was reduced in the papillary dermis of this patient with acquired cutis laxa.[1]


  1. Abnormalities of fibrillin in acquired cutis laxa. Lebwohl, M.G., Schwartz, E., Jacobs, L., Lebwohl, M., Sakai, L., Fleischmajer, R. J. Am. Acad. Dermatol. (1994) [Pubmed]
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