Congenital hypotrichosis and milia with spontaneous regression during adolescence or Oley syndrome: a variant of Bazex-Dupre-Christol syndrome.

Background. We report a family presenting the syndrome initially described by Oley characterized by congenital profus milia and hypotrichosis that reg ress during adolescence. Case report. A female infant with severe congenital hypotrichosis had profu s milia involving the entire face. The remainder of the physical examinatio n was normal. The mother had normal skin and hair but indicated she had had the same signs as a child. The patient's condition regressed from the age of lo to 15 years but she has undergone several surgical resections for bas ocellular carcinoma since the age of 20. The maternal grandfather had spont aneously regressive typical follicular atrophodermia involving the back and the hands and also had several milium grains and several basocellular carc inomas. He had never presented hypotrichosis. Discussion. Oley syndrome is defined as an association of congenital hypotr ichosis and milia spontaneously regressive during adolescence. The symptoms presented by our patient and her mother are similar to this genodermatosis . Concomitant hypotrichosis, milia, basocellular carcinomas and follicular atrophodermia define the Bazex-Dupre-Christol syndrome. The grandfather's c ondition would be closer to this syndrome than Oley syndrome despite the sp ontaneous regression of certain anomalies during adolescence. This family s tudy would suggest that the Bazex-Dupre-Christol syndrome and the Oley synd rome are two variants of the same condition.