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遗传性屈曲指是常染色体显性遗传的纤维瘤病的一种罕见类型,男女均可发生,始于童年,可涉及两手的小指,表现勾近端指关节屈曲,而掌指关节正常;有时也可发生于其它手指。它可使手形变得很像麻风的爪状手。因为在麻风流行区麻风是爪状手的主要原因,所以鉴别这种病很重要,以免将其误诊为麻风。作者在印度曾见过两例此种病人,开始都被怀疑为麻风。这两例都是出生时就两手小指屈曲畸形,1例为16岁的女孩,另1例为45岁的男子,后者的两手环指也屈曲;两人都不曾有浅色斑、感觉障碍或神经痛的历史。该女孩的父亲和男子的母亲也有类似的毛病。当地医生曾怀疑这二人为麻风,小女孩还用过6个月的抗麻风药物。检查见女孩两手的小指及男人两手的小指及环指近端指关节呈固定性屈曲,感觉正常,无神经肿大。
Hereditary buckling refers to a rare type of autosomal dominant fibromatosis that occurs both in men and women, beginning in childhood and involving the little fingers of both hands, presenting with proximal flexor knuckles and normal metacarpophalangeal joints; sometimes It can happen to other fingers as well. It can make hand shape like a leprous claw hand. Because leprosy is the major cause of claw-like hand in the endemic area of leprosy, identification of the disease is important to avoid misdiagnosing it as leprosy. The author has seen two such patients in India and was initially suspected of leprosy. Both cases were born flexed deformities of the little fingers, one was a 16-year-old girl and the other was a 45-year-old man whose ring fingers were also flexed; both had no light spots and sensory disturbances Or neuralgia history. The girl’s father and the mother’s mother have similar problems. The local doctors suspected the two were leprosy and the little girl had used anti-leprosy drugs for six months. Check see the girl’s hands and pinky both hands of the fingers and ring finger proximal fixed flexion, feeling normal, no neurological enlargement.