Picnodisostose de Maroteaux-Lamy associada a malformações genitais externas: a case report

Abstract

The case of a seven-years-old girl with features of Maroteaux-Lamy's pycnodysostosis associated with external genital envolvement is reported. Considering the apparent and gradual increase of the clinical traits in this condition, this case may represent some contribution to its nosography. Additionally dermatoglyps technique and analysis were done which peculiar results are discussed.