Restrictive dermopathy: a disorder of skin differentiation with abnormal integrin expression
Corresponding Author
John C. S. Dean
Departments of Medical Genetics, University of Aberdeen Medical School, Harrow, Middlesex, UK
Department of Medical Genetics, Medical School, Foresterhill, Aberdeen AB9 2ZD, Scotland, UKSearch for more papers by this authorE. S. Gray
Pathology, University of Aberdeen Medical School, Harrow, Middlesex, UK
Search for more papers by this authorK. N. Stewart
Medicine and Therapeutics, University of Aberdeen Medical School, Harrow, Middlesex, UK
Search for more papers by this authorT. Brown
Departments of Medical Genetics, University of Aberdeen Medical School, Harrow, Middlesex, UK
Search for more papers by this authorD. J. Lloyd
Neunatal Unit, Aberdeen Maternity Hospital, Foresterhill, Aberdeen, Harrow, Middlesex, UK
Search for more papers by this authorN. C. Smith
Obstetrics and Gynaecology, University of Aberdeen Medical School, Harrow, Middlesex, UK
Search for more papers by this authorF. M. Pope
Dermatology Research Group, Clinical Research Centre, Northwick Park Hospital, Harrow, Middlesex, UK
Search for more papers by this authorCorresponding Author
John C. S. Dean
Departments of Medical Genetics, University of Aberdeen Medical School, Harrow, Middlesex, UK
Department of Medical Genetics, Medical School, Foresterhill, Aberdeen AB9 2ZD, Scotland, UKSearch for more papers by this authorE. S. Gray
Pathology, University of Aberdeen Medical School, Harrow, Middlesex, UK
Search for more papers by this authorK. N. Stewart
Medicine and Therapeutics, University of Aberdeen Medical School, Harrow, Middlesex, UK
Search for more papers by this authorT. Brown
Departments of Medical Genetics, University of Aberdeen Medical School, Harrow, Middlesex, UK
Search for more papers by this authorD. J. Lloyd
Neunatal Unit, Aberdeen Maternity Hospital, Foresterhill, Aberdeen, Harrow, Middlesex, UK
Search for more papers by this authorN. C. Smith
Obstetrics and Gynaecology, University of Aberdeen Medical School, Harrow, Middlesex, UK
Search for more papers by this authorF. M. Pope
Dermatology Research Group, Clinical Research Centre, Northwick Park Hospital, Harrow, Middlesex, UK
Search for more papers by this authorAbstract
Dean JCS, Gray ES, Stewart KN, Brown T, Lloyd DJ, Smith NC, Pope FM. Restrictive dermopathy: a disorder of skin differentiation with abnormal integrin expression.
Clin Genet 1993: 44: 287–291. © Munksgaard, 1993
Clinical features and histological findings in two sibs who died from restrictive dermopathy in the neonatal period are described. Fibroblasts cultured from a skin biopsy from the second sib and fibroblasts from normal neonatal skin were studied using monoclonal antibodies to visualise integrin subunits by immunocytochemistry. Restrictive dermopathy fibroblasts displayed increased expression of the alpha-1 and alpha-2 subunits of integrin, those responsible for collagen binding. The increase was not matrix dependent. Integrins may play an important role in tissue differentiation, and our findings support the hypothesis that restrictive dermopathy is a disorder of skin differentiation.
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