Caccavale Stefano, Bove Domenico, Bove Rocco M, LA Montagna Maddalena
Department of Mental and Physical Health and Preventive Medicine, Second University of Naples, Naples, Italy.
Fusis Association for Research in Child and Adolescent Neuropsychiatry, Alvignano, Italy.
G Ital Dermatol Venereol. 2017 Feb;152(1):58-65. doi: 10.23736/S0392-0488.16.05083-5. Epub 2016 Mar 22.
This article explores three neurocutaneous syndromes (NCSs), i.e. genetic disorders producing developmental abnormalities of the skin and an increased risk of neurological complications. In this review, different aspects of ataxia telangiectasia, Menkes kinky hair disease and neurocutaneous melanosis are examined: clinical features, genetic defect, mutation spectrum, pathogenesis, and neurobiological basis; indications for clinical practice are also provided to the readers. The aim of this review is to stress the importance of cooperation among dermatologists, neurologists and psychiatrists, in order to provide patients suffering from these diseases with timely diagnosis and targeted treatments.
本文探讨了三种神经皮肤综合征(NCSs),即导致皮肤发育异常和神经并发症风险增加的遗传性疾病。在本综述中,对共济失调毛细血管扩张症、门克斯卷发综合征和神经皮肤黑变病的不同方面进行了研究:临床特征、基因缺陷、突变谱、发病机制和神经生物学基础;还向读者提供了临床实践的指征。本综述的目的是强调皮肤科医生、神经科医生和精神科医生之间合作的重要性,以便为患有这些疾病的患者提供及时的诊断和针对性治疗。