[Congenital generalized cutis laxa].
Congenital cutis laxa is a rare disorder of the elastic tissue in which lax skin gives a premature senile appearance. We report a new case of this disease associated with craniosynostosis. In the literature, the genetics of cutis laxa are not clear. Fleischmajer and Matus (18) considers an inherited autosomal recessive or an incomplete autosomal dominant trait, Mehregan (33) reports an autosomal recessive mode of transmission and Byers (10) an X-linked form. Among these cases, the most serious visceral involvement is the development of pulmonary emphysema. However, in cutis laxa with development retardation, variety of minor injury of the skeleton, the prognosis seems appears to be better.
D003483:Cutis Laxa / Q000151:congenital* / Q000175:diagnosis / Q000503:physiopathology; D003937:Diagnosis, Differential; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D011379:Prognosis
Cutis laxa généralisée congénitale.