Department of Clinical Medicine and Immunological Sciences, University of Siena, Siena, Italy.
G Ital Dermatol Venereol. 2009 Oct;144(5):617-9.
The sebaceous nevus syndrome (SNS) is characterized by the presence of a sebaceous nevus and extracutaneous abnormalities, usually involving organs derived from the neuroectoderm. The authors report the case of a 22 year-old patient with a systematized sebaceous nevus on the forehead and scalp and a history of developmental delay and severe seizure disorders. The father of the patient also indicated prior surgery to correct an ocular coloboma and prior removal of a squamous cell epithelioma of the tongue. Firstly described by Gustav Schimmelpenning in 1957, SNS, also known as 'Schimmelpenning-Feuerstein-Mims syndrome', is one of the six different types of epidermal nevus syndromes. In patients with SNS the risk of developing neoplasms is higher than that of the general population. The particularity of our case lies in the late diagnosis. The authors believe that early diagnosis and awareness of the potential presence of neoplasms are important for those involved in the care of patients with SNS.
皮脂腺痣综合征(SNS)的特征是存在皮脂腺痣和皮肤外异常,通常涉及源自神经外胚层的器官。作者报告了一例 22 岁患者的病例,其额部和头皮有系统性皮脂腺痣,并有发育迟缓及严重癫痫发作病史。患者的父亲还表示曾接受过眼部虹膜缺损矫正手术和舌部鳞状细胞上皮瘤切除术。SNS 由 Gustav Schimmelpenning 于 1957 年首次描述,也称为“Schimmelpenning-Feuerstein-Mims 综合征”,是六种不同类型的表皮痣综合征之一。SNS 患者的肿瘤发病风险高于一般人群。我们的病例的特殊性在于其诊断较晚。作者认为,对于 SNS 患者的护理人员而言,早期诊断和认识到潜在肿瘤的存在是很重要的。