title: [Congenital hypotrichosis and milia with spontaneous regression during adolescence or Oley syndrome: a variant of Bazex-Dupré-Christol syndrome].
abstract: BACKGROUND
  We report a family presenting the syndrome initially described by Oley characterized by congenital profus milia and hypotrichosis that regress during adolescence.
  
  
  METHODS
  A female infant with severe congenital hypotrichosis had profus milia involving the entire face. The remainder of the physical examination 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 10 to 15 years but she has undergone several surgical resections for basocellular carcinoma since the age of 20. The maternal grandfather had spontaneously regressive typical follicular atrophodermia involving the back and the hands and also had several milium grains and several basocellular carcinomas. He had never presented hypotrichosis.
  
  
  CONCLUSIONS
  Oley syndrome is defined as an association of congenital hypotrichosis 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-Dupré-Christol syndrome. The grandfather's condition would be closer to this syndrome than Oley syndrome despite the spontaneous regression of certain anomalies during adolescence. This family study would suggest that the Bazex-Dupré-Christol syndrome and the Oley syndrome are two variants of the same condition.
authors.affiliation: Service de Dermatologie, Hôpital Sainte-Marguerite, 13009 Marseille.
authors.forename: V
authors.initials: V
authors.lastname: Andreani
authors.forename: M
authors.initials: M
authors.lastname: Richard
authors.forename: G
authors.initials: G
authors.lastname: Folchetti
authors.forename: S
authors.initials: S
authors.lastname: Varennes
authors.forename: N
authors.initials: N
authors.lastname: Philip
authors.forename: J J
authors.initials: JJ
authors.lastname: Grob
mesh_terms: D000293:Adolescent; D000328:Adult; D002280:Basal Cell Carcinoma / Q000175:diagnosis / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007039:Hypotrichosis / Q000175:diagnosis / Q000235:genetics*; D007223:Infant; D008883:Miliaria / Q000175:diagnosis / Q000235:genetics*; D009364:Neoplasm Recurrence, Local / Q000175:diagnosis / Q000235:genetics; D010375:Pedigree; D012075:Remission, Spontaneous; D012878:Skin Neoplasms / Q000175:diagnosis / Q000235:genetics; D013577:Syndrome