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[Essential primary cutis verticis gyrata]

Letícia K Schenato1, Tatiane Gil, Lauro A Carvalho

  • 1Faculdade Federal de Ciências Médicas de Porto Alegre (FFFCMPA), RS, Brazil.

Jornal De Pediatria
|December 4, 2003
PubMed
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This report details a rare case of primary Cutis verticis gyrata in a child, characterized by scalp skin hypertrophy. The condition presented without associated neurological or ophthalmological issues, marking a unique pediatric presentation.

Area of Science:

  • Dermatology
  • Pediatric Case Reports

Background:

  • Cutis verticis gyrata (CVG) is a rare condition characterized by scalp folding resembling cerebral gyri.
  • Primary CVG is typically associated with genetic factors and lacks underlying systemic abnormalities.
  • Secondary CVG can be linked to various neurological, ophthalmological, or endocrine disorders.

Purpose of the Study:

  • To present a rare case of essential primary Cutis verticis gyrata in a pediatric patient.
  • To highlight the diagnostic criteria and differential diagnoses for primary CVG in children.
  • To emphasize the significance of this case as the first reported instance in a child.

Main Methods:

  • Clinical examination of a nine-year-old boy presenting with extensive scalp hypertrophy.
  • Neurological and ophthalmological assessments to rule out associated disorders.

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  • Family history evaluation to assess for genetic predisposition.

    • Main Results:

    • A nine-year-old boy exhibited significant scalp skin hypertrophy with a cerebriform appearance.
    • No underlying neurological or ophthalmological abnormalities were identified.
    • No similar cases were reported within the patient's family history.

    Conclusions:

    • The diagnosis of primary Cutis verticis gyrata was confirmed based on scalp thickening and the absence of associated abnormalities.
    • Essential primary CVG is a rare diagnosis in pediatric patients.
    • Differential diagnoses, including secondary causes like nevus, pachydermoperiostosis, acromegaly, and inflammatory scalp diseases, were considered and excluded.