Kathuria Sushruta, Arora Shikha, Ramesh V
Dermatol Online J. 2012 Sep 15;18(9):11.
Sjögren-Larsson syndrome (SLS) is a congenital ichthyotic disorder with spasticity. We describe a case of a 5-year-old boy with SLS diagnosed clinically based on congenital ichythosis, quadriplegia, and mental retardation. The child responded well to emollients and antihistamines. His quadriplegia was managed by aggressive physiotherapy and mental retardation by stimulation techniques. After a 3-year follow up, significant improvement was seen in his motor and mental disability. This case highlights the importance of clinical diagnosis and early intervention for such a disabling disorder.
舍格伦-拉尔松综合征(SLS)是一种伴有痉挛的先天性鱼鳞病。我们描述了一例5岁男孩患舍格伦-拉尔松综合征的病例,该病例根据先天性鱼鳞病、四肢瘫痪和智力发育迟缓在临床上被诊断出来。该患儿对润肤剂和抗组胺药反应良好。他的四肢瘫痪通过积极的物理治疗进行处理,智力发育迟缓则通过刺激技术进行处理。经过3年的随访,他的运动和智力残疾有了显著改善。这个病例凸显了对于这种致残性疾病进行临床诊断和早期干预的重要性。