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., ...Daha Fazla

JOURNAL OF DERMATOLOGY, cilt.30, no.3, ss.222-225, 2003 (SCI İndekslerine Giren Dergi) identifier


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.