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Netherton Syndrome Associated with Idiopathic Congenital Hemihypertrophy
Author(s) -
Yerebakan Özlem,
Uğuz Ayşen,
Keser İbrahim,
Lüleci Güven,
Çiftçioğlu Mehmet Akif,
Başaran Erdal,
Alpsoy Erkan
Publication year - 2002
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1046/j.1525-1470.2002.00098.x
Subject(s) - medicine , hemihypertrophy , dermatology , genodermatosis , pediatrics , pathology , biochemistry , chemistry , gene
Netherton syndrome is a rare genodermatosis comprised of anichthyosiform dermatitis, hair shaft defects, and atopic features. Other problems associated with Netherton syndrome are delayed growth and development, immune abnormalities, recurrent infections, and intermittent aminoaciduria. We describe an 18‐month‐old girl with Netherton syndrome who had idiopathic congenital hemihypertrophy on her right side with contralateral benign nephromegaly in addition to the characteristic clinical signs of the syndrome. To our knowledge, this is the first case of Netherton syndrome associated with idiopathic congenital hemihypertrophy to be reported.