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马富西综合征:一种间充质发育异常和多发性肿瘤综合征。

Maffucci's syndrome: amesenchymal dysplasia and multiple tumour syndrome.

作者信息

Loewinger R J, Lichtenstein J R, Dodson W E, Eisen A Z

出版信息

Br J Dermatol. 1977 Mar;96(3):317-22. doi: 10.1111/j.1365-2133.1977.tb06145.x.

Abstract

A case of Maffucci's syndrome with cutaneous, bony and neurological complications is reported. The patient had lymphangiomatosis at birth and developed multiple cutaneous haemangiomas and osteochondromas during childhood. She also developed multiple neurological defects, including cranial nerve palsies due to an intracranial osteochondroma. The occurence of mesodermal dysplasias and neoplasias in the Maffucci syndrome is emphasized, and it is suggested that there is a close relationship between this disorder and Ollier's disease (multiple enchondromatosis).

摘要

报告了一例伴有皮肤、骨骼和神经并发症的马富西综合征病例。该患者出生时即患有淋巴管瘤病,儿童期出现多发性皮肤血管瘤和骨软骨瘤。她还出现了多种神经缺陷,包括因颅内骨软骨瘤导致的颅神经麻痹。强调了马富西综合征中中胚层发育异常和肿瘤的发生情况,并提示该疾病与奥利尔病(多发性内生软骨瘤病)之间存在密切关系。

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