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[Acanthosis nigricans in children and Crouzon syndrome].
Ann Dermatol Venereol. 2014 Nov; 141(11):685-8.AD

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.

PATIENTS AND 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.

DISCUSSION

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+Show Affiliations

Service de dermatologie, CHU/CHR Clémenceau, avenue Georges-Clemenceau, 14033 Caen cedex 9, France. Electronic address: marionlagaude@neuf.fr.Service de dermatologie, CHU/CHR Clémenceau, avenue Georges-Clemenceau, 14033 Caen cedex 9, France.Service de réanimation médico-chirurgicale pédiatrique, CHU, avenue de la Côte-de-Nacre, 14000 Caen, France.Service de génétique, CHU, avenue de la Côte-de-Nacre, 14000 Caen, France.Service de neurochirurgie pédiatrique, hôpital Necker-Enfants-Malades, 149, rue de Sèvres, 75015 Paris, France.Service de dermatologie, hôpital Necker-Enfants-Malades, 149, rue de Sèvres, 75015 Paris, France.Service de dermatologie, CHU/CHR Clémenceau, avenue Georges-Clemenceau, 14033 Caen cedex 9, France.Service de dermatologie, CHU/CHR Clémenceau, avenue Georges-Clemenceau, 14033 Caen cedex 9, France.

Pub Type(s)

Case Reports
English Abstract
Journal Article

Language

fre

PubMed ID

25442473

Citation

Lagaude, M, et al. "[Acanthosis Nigricans in Children and Crouzon Syndrome]." Annales De Dermatologie Et De Venereologie, vol. 141, no. 11, 2014, pp. 685-8.
Lagaude M, Barreau M, Jokic M, et al. [Acanthosis nigricans in children and Crouzon syndrome]. Ann Dermatol Venereol. 2014;141(11):685-8.
Lagaude, M., Barreau, M., Jokic, M., Gerard, M., DiRocco, F., Hadj-Rabia, S., Dompmartin, A., & Verneuil, L. (2014). [Acanthosis nigricans in children and Crouzon syndrome]. Annales De Dermatologie Et De Venereologie, 141(11), 685-8. https://doi.org/10.1016/j.annder.2014.06.020
Lagaude M, et al. [Acanthosis Nigricans in Children and Crouzon Syndrome]. Ann Dermatol Venereol. 2014;141(11):685-8. PubMed PMID: 25442473.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Acanthosis nigricans in children and Crouzon syndrome]. AU - Lagaude,M, AU - Barreau,M, AU - Jokic,M, AU - Gerard,M, AU - DiRocco,F, AU - Hadj-Rabia,S, AU - Dompmartin,A, AU - Verneuil,L, Y1 - 2014/08/07/ PY - 2014/03/12/received PY - 2014/05/10/revised PY - 2014/06/19/accepted PY - 2014/12/3/entrez PY - 2014/12/3/pubmed PY - 2015/7/22/medline KW - Acanthosis nigricans KW - Craniosténose KW - Craniosynostosis KW - Crouzon (syndrome de) KW - Crouzon syndrome KW - FGFR3 SP - 685 EP - 8 JF - Annales de dermatologie et de venereologie JO - Ann Dermatol Venereol VL - 141 IS - 11 N2 - BACKGROUND: Crouzon syndrome with acanthosis nigricans is a rare form of Crouzon syndrome in which craniosynostosis and facial dysmorphism are associated with acanthosis nigricans. PATIENTS AND 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. DISCUSSION: 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. SN - 0151-9638 UR - https://wwww.unboundmedicine.com/medline/citation/25442473/[Acanthosis_nigricans_in_children_and_Crouzon_syndrome]_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0151-9638(14)00491-8 DB - PRIME DP - Unbound Medicine ER -