Schmitt A M, Bories A, Baran R
Service de dermatologie Hôpital Rangueil Toulouse.
Ann Dermatol Venereol. 1997;124(3):233-6.
The multiple exostosis syndrome is a rare disease transmitted by autosomal dominant inheritance. Bone growth projecting outward from the long bones is observed in multiple localizations during growth. Prognosis of this benign disease is worsened by the possibility of chondrosarcoma. We report three cases of subungueal exostoses observed in children revealing hereditary exostosis.
Clinical examination evidenced progressive deformation of several fingers. In all three patients. There was subungueal tumefaction raising a fissured nail with longitudinal crests. In two cases there were also skeletal deformations. Radiograms showed multiple exostoses of the long bones in all three cases and in one a tumefaction of the scapula in addition to visualizing the subungueal exostoses. There was a family history of such manifestations in all cases.
Subungueal exostosis is frequent in young adults, usually located in the large toe. These three observations were particularly interesting due to the subungueal localization during the first decade of life. Such localization are rare and usually concern several fingers.
多发性外生骨疣综合征是一种罕见的常染色体显性遗传病。在生长过程中，可在多个部位观察到从长骨向外突出的骨生长。这种良性疾病的预后因软骨肉瘤的发生可能性而恶化。我们报告了3例在儿童中观察到的甲下外生骨疣病例，提示遗传性外生骨疣。
临床检查发现几个手指有进行性变形。在所有3例患者中，均有甲下肿胀，使指甲出现裂纹并伴有纵向嵴。2例患者还存在骨骼变形。X线片显示所有3例患者长骨均有多发性外生骨疣，其中1例除可见甲下外生骨疣外，肩胛骨还有肿胀。所有病例均有此类表现的家族史。
甲下外生骨疣在年轻人中较为常见，通常位于大脚趾。这3例病例特别有趣，因为甲下病变发生在生命的第一个十年。这种定位很罕见，通常累及几个手指。
