Report of a Turkish child with Sjogren-Larsson syndrome associated with peripheral nerve involvement


AKDENİZ N. , Çalka Ö., Anlar Ö., Akbayram S., Çaksen H., Metin A., ...More

JOURNAL OF DERMATOLOGY, vol.30, no.3, pp.222-225, 2003 (Journal Indexed in SCI) identifier

  • Publication Type: Article / Article
  • Volume: 30 Issue: 3
  • Publication Date: 2003
  • Doi Number: 10.1111/j.1346-8138.2003.tb00375.x
  • Title of Journal : JOURNAL OF DERMATOLOGY
  • Page Numbers: pp.222-225

Abstract

Sjogren-Larsson syndrome is a rare hereditary neurocutaneous disorder characterized by ichthyosis, spastic di- or tetra-plegia, and mild to moderate mental retardation. In this article, we present a nine-year-old girl with the classical features of the syndrome associated with peripheral nerve involvement because of its rare presentation. To the best of our knowledge, only three cases of Sjogren-Larsson syndrome with peripheral nerve involvement have been previously reported in the literature. We assume that Sjogren-Larsson syndrome involves extensive disorders of the ectodermal tissues, including the peripheral nerves as well as the skin and the central nervous system.