[Keratotic neurocutaneous syndromes].
OBJECTIVE
To review current knowledge of etiologic, clinic, diagnostic and therapeutic aspects of ichthyotic diseases with neurologic manifestations.


METHODS
Classic keratotic neurocutaneous syndromes including Sjögren-Larsson syndrome, trichotyodystrophy, KID (keratitis, ichthyosis and deafness) syndrome and Rud syndrome, are reviewed. Furthermore, we pay attention to syndromes whose description and study are of current importance: cardiofaciocutaneous syndrome, neutral lipids storage disease with ichthyosis, multiple sulphatase deficiency disease and peroxisomal disorders.


CONCLUSIONS
Keratotic neurocutaneous syndromes knowledge will help to improve its diagnosis and therapeutic approach.
Tercedor
J
J
Sección de Dermatología, Hospital Universitario Virgen de las Nieves, Granada, España.
García
A
A
D000326:Adrenoleukodystrophy / Q000175:diagnosis; D002806:Chondrodysplasia Punctata / Q000175:diagnosis; D003638:Deafness / Q000150:complications; D006801:Humans; D007057:Ichthyosis / Q000150:complications / Q000175:diagnosis*; D018901:Peroxisomal Disorders / Q000175:diagnosis*; D012035:Refsum Disease / Q000175:diagnosis; D016111:Sjogren-Larsson Syndrome / Q000175:diagnosis; D013577:Syndrome