RESTRICTIVE DERMOPATHY - A DISORDER OF SKIN DIFFERENTIATION WITH ABNORMAL INTEGRIN EXPRESSION

Clinical features and histological findings in two sibs who died from restrictive dermopathy in the neonatal period are described. Fibroblas ts cultured from a skin biopsy from the second sib and fibroblasts fro m normal neonatal skin were studied using monoclonal antibodies to vis ualise integrin subunits by immunocytochemistry. Restrictive dermopath y fibroblasts displayed increased expression of the alpha-1 and alpha- 2 subunits of integrin, those responsible for collagen binding. The in crease was not matrix dependent. Integrins may play an important role in tissue differentiation, and our findings support the hypothesis tha t restrictive dermopathy is a disorder of skin differentiation.