title: [Acanthosis nigricans in children and Crouzon syndrome].
abstract: BACKGROUND
  Crouzon syndrome with acanthosis nigricans is a rare form of Crouzon syndrome in which craniosynostosis and facial dysmorphism are associated with acanthosis nigricans.
  
  
  METHODS
  Cutaneous examination of a 9-year-old child presenting bicoronal craniosynostosis revealed acanthosis nigricans of the cervical, axillar, inguinal and popliteal regions which appeared at the age of two. He had a dysmorphic face including a large forehead, hypertelorism, mid-face hypoplasia, prognathism and low-set ears. These clinical anomalies suggested a case of Crouzon syndrome with acanthosis nigricans, which was later confirmed by the finding of a mutation in the FGFR3 gene.
  
  
  CONCLUSIONS
  Acanthosis nigricans in children is often a cutaneous marker of insulin resistance. However, it may also form part of diverse diseases, notably those of genetic origin. The association of craniosynostosis and acanthosis nigricans allows incrimination of the FGFR3 gene from the outset and diagnosis of Crouzon syndrome with acanthosis nigricans. In the present case, dermatological examination allowed an aetiology of craniosynostosis to be determined.
authors.affiliation: Service de dermatologie, CHU/CHR Clémenceau, avenue Georges-Clemenceau, 14033 Caen cedex 9, France. Electronic address: marionlagaude@neuf.fr.
authors.forename: M
authors.initials: M
authors.lastname: Lagaude
authors.affiliation: Service de dermatologie, CHU/CHR Clémenceau, avenue Georges-Clemenceau, 14033 Caen cedex 9, France.
authors.forename: M
authors.initials: M
authors.lastname: Barreau
authors.affiliation: Service de réanimation médico-chirurgicale pédiatrique, CHU, avenue de la Côte-de-Nacre, 14000 Caen, France.
authors.forename: M
authors.initials: M
authors.lastname: Jokic
authors.affiliation: Service de génétique, CHU, avenue de la Côte-de-Nacre, 14000 Caen, France.
authors.forename: M
authors.initials: M
authors.lastname: Gerard
authors.affiliation: Service de neurochirurgie pédiatrique, hôpital Necker-Enfants-Malades, 149, rue de Sèvres, 75015 Paris, France.
authors.forename: F
authors.initials: F
authors.lastname: DiRocco
authors.affiliation: Service de dermatologie, hôpital Necker-Enfants-Malades, 149, rue de Sèvres, 75015 Paris, France.
authors.forename: S
authors.initials: S
authors.lastname: Hadj-Rabia
authors.affiliation: Service de dermatologie, CHU/CHR Clémenceau, avenue Georges-Clemenceau, 14033 Caen cedex 9, France.
authors.forename: A
authors.initials: A
authors.lastname: Dompmartin
authors.affiliation: Service de dermatologie, CHU/CHR Clémenceau, avenue Georges-Clemenceau, 14033 Caen cedex 9, France.
authors.forename: L
authors.initials: L
authors.lastname: Verneuil
mesh_terms: D000052:Acanthosis Nigricans / Q000175:diagnosis* / Q000235:genetics; D002648:Child; D003394:Craniofacial Dysostosis / Q000175:diagnosis* / Q000235:genetics; D003398:Craniosynostoses / Q000175:diagnosis; D006801:Humans; D006972:Hypertelorism / Q000175:diagnosis; D008297:Male; D009154:Mutation / Q000235:genetics; D011378:Prognathism / Q000175:diagnosis; D051498:Receptor, Fibroblast Growth Factor, Type 3 / Q000235:genetics
chemical_list: C496365:FGFR3 protein, human; D051498:Receptor, Fibroblast Growth Factor, Type 3
keywords: Acanthosis nigricans; Craniosténose; Craniosynostosis; Crouzon (syndrome de); Crouzon syndrome; FGFR3