Fehlow P, Tennstedt A
Psychiatr Neurol Med Psychol (Leipz). 1975 Jun;27(6):341-7.
The observations made on a 27-year-old patient with ichthyosis, debility, psychoinfantilism, and Little's syndrome are reported. Clinically, the condition of the skin is similar to that associated with ichthyosis vulgaris. Histologically, however, the picture is largely that of ichthyosis congenita, the condition being even more pronounced than that of ichthyosis congenita mitis. This particular case may be placed within the class of transitional forms of hereditary ichthyoses, and it may thus be proved that the Sjögren-Larsson syndrome also has its 'formes de passage'. In the etiological determination of oligophrenias and infantile cerebral motor disorders it is always necessary to give attention to the probable presence of ichthyosiform conditions of the skin.
报告了对一名患有鱼鳞病、虚弱、精神幼稚症和利特尔综合征的27岁患者的观察结果。临床上,皮肤状况与寻常型鱼鳞病相关的状况相似。然而,组织学上,其表现主要是先天性鱼鳞病的表现,这种情况比重症先天性鱼鳞病更为明显。这个特殊病例可能属于遗传性鱼鳞病的过渡形式类别,因此可以证明舍格伦-拉尔松综合征也有其“过渡形式”。在智力发育迟缓及婴儿期脑性运动障碍的病因判定中,始终有必要关注皮肤鱼鳞病样状况可能的存在。