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Japanese

ACROPATHIE ULCÉROMUTILANTE FAMILIALE DE THÉVENARD: FAMILIÄRE AKROOSTEOLYSE Junji TANIFUJI 1 , Teruya HIRAYAMA 1 , Akitoshi ARIGA 1 , Morito NAGAI 1 1Department of Dermatology, University of Hokkaido, Faculty of Medicine Keyword: e pp.572,579-584
Published Date 1969/6/1
DOI https://doi.org/10.11477/mf.1412200512
  • Abstract
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 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.


Copyright © 1969, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1324 印刷版ISSN 0021-4973 医学書院

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