Loth M, Waibel M, Albrecht G
Dermatologische Abteilung des Krankenhauses Spandau.
Hautarzt. 1998 Jun;49(6):505-8. doi: 10.1007/s001050050779.
The Christ-Siemens-Touraine syndrome (CST syndrome) is the rare an- or hypohidrotic form of the ectodermal dysplasia. During embryonic development, the formation and maturation of the central nervous system and the epithelia of the sense organs, skin, teeth, hair (and less frequently the nails) are disturbed. In addition to the cosmetic impairment, insufficient or absent secretion of sweat and sebum is particularly discomforting as it leads to extreme heat intolerance. The decreased number of apocrine sweat glands and mucous glands in the upper nasopharynx results in diminished resistance to respiratory infections. We describe typical features of the disease in a 60 year old female patient, distinguishing this disease from other forms of ectodermal dysplasia. Diagnostic criteria as well as therapeutic options are discussed.
克里斯蒂-西门子-图赖讷综合征（CST综合征）是外胚层发育不良中罕见的无汗或少汗型。在胚胎发育过程中，中枢神经系统以及感觉器官、皮肤、牙齿、毛发（指甲较少见）的上皮形成和成熟受到干扰。除了外貌受损外，汗液和皮脂分泌不足或缺乏尤其令人不适，因为这会导致极度不耐热。鼻咽上部顶泌汗腺和黏液腺数量减少导致对呼吸道感染的抵抗力下降。我们描述了一名60岁女性患者的该疾病典型特征，将这种疾病与其他形式的外胚层发育不良区分开来。并讨论了诊断标准和治疗选择。
