Acropathie ulcéromutilante familiale de Thévenard（familiäre Akroosteolyse）の1例

ACROPATHIE ULCÉROMUTILANTE FAMILIALE DE THÉVENARD: FAMILIÄRE AKROOSTEOLYSE

I．はじめに

　足穿孔症（Malum perforans pedis）は，古くから癩，糖尿病，脊髄癆，脊髄空洞症などの中枢神経または末梢神経の障害として生ずることが知られているが，近年，特別な前駆疾患なしに骨変化を伴つて生ずる症例が，相次いで報告されている。

　海外ではすでに19世紀の終りに，本症に属する疾患が記載されているが，Kienböck^1）は，二分脊椎に起因するものをTrophopathia pedismyelodysplasticaと命名し，1つのclinicalentityとした。一方Thévenard^2）は，臨床的にはほぼ同じ像を呈するが必ずしも二分脊椎を伴わず，家族性に発生するものをAcropathie ulcéromutilante familialeとして分離した。さらにBureau et Barriére^3）は，ThévenardのAcropathieとほぼ同様の病像を呈する，非遺伝性のものにAcropathies ulcéromutilantes pseudosyringomyeliques non familiales des membres inférieursの名称を与えている。

　A 23-year-old business girl appeared at first on May 13. 1967, complaining of deformity of both feet, ulceration of the right sole, and hypesthesia of the extremities.

　Her parents were consanguineous, and her elder sister had the same disorder. She has not felt pain on trauma since 6 years of age, and has had perniolike lesions and darkening of the right first toe since 10 years of age, which was diagnosed as spontaneous gangrene. At the age of 15, deformity and swelling of the right foot were increased and similar lesions were found also on the left foot. Sequestrectomy of the left first toe at the age of 17, amputation of all the toes of the right foot the next year, and right sympathectomy at the age of 19 were performed. The ulcer of the right sole showed a tendency to enlargement. At the first visit, the right foot was woodenhard and swollen, the distal part of which was lost. On the sole the ulcer was 4cm in diameter, surrounded by tylosislike keratosis, the base of which was composed of loose granular granuloma. Marked swelling and deformity of the left 1st and 2nd toes and fistulason the dorsal and plantar surfaces of the base of the 3 rd toe were seen Roentgenogram of bone showed loss of the distal part of the right Lisfranc joint and lysis, caries, fracture or periostosislike change in the metatarsal or proximal phalangeal bones of the 2nd to 5th toes. Although decreased patellar and Achilles tendon reflexes, hypesthesia of the lower legs and forearms, anesthesia of the feet, increased sedimentation rate and high ASLO-titer were proved, pathological reflexes, disturbances of urination and evacuation, and abnormalities in e.m.g. and in roentgenogram of the vertebra were not found.