进行性骨化性纤维发育不良

Fibrodysplasia ossificants progressiva.

作者信息

Hentzer B, Jacobsen H H, Asboe-Hansen G

出版信息

Scand J Rheumatol. 1977;6(3):161-71. doi: 10.3109/03009747709095443.

Abstract

Two cases of fibrodysplasia ossificans progressiva are reported. Both patients were females and had suffered from the disease since birth. The characteristic anomalies of great toes and thumbs associated with multiple ectopic ossifications of the connective tissue are described. Fasciae, tendons, ligaments, and joint capsules of the proximal parts of the extremities and the dorsal aspect of the trunk were involved. The muscle atrophy was probably a secondary phenomenon.

摘要

报告了两例进行性骨化性纤维发育不良病例。两名患者均为女性，自出生起就患有该病。描述了与结缔组织多处异位骨化相关的大脚趾和拇指的特征性异常。四肢近端部分的筋膜、肌腱、韧带和关节囊以及躯干的背侧均受累。肌肉萎缩可能是一种继发现象。