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[伴有皮肤色素沉着、多毛、水肿及λ型免疫球蛋白A升高的多发性神经炎中的显著内分泌异常:一例报告]

[Marked endocrine abnormalities in polyneuritis with skin hyperpigmentation, hypertrichosis, edema and increased lambda type immunoglobulin A: a case report].

作者信息

Yoshida T, Kumakura H, Asato H, Sugano J, Yanagisawa H

出版信息

Nihon Naibunpi Gakkai Zasshi. 1983 Sep 20;59(9):1237-43. doi: 10.1507/endocrine1927.59.9_1237.

DOI:10.1507/endocrine1927.59.9_1237
PMID:6416899
Abstract

A syndrome which is known as plasma cell dyscrasia with polyneuropathy and various endocrine manifestations or plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, abnormal M protein and skin changes is very interesting because this syndrome has miscellaneous manifestations such as skin hyperpigmentation, hypertrichosis, polyneuropathy, M protein abnormality, plasma cell dyscrasia and endocrine disturbances. Miscellaneous endocrine abnormalities which have not been described so far are reported here. A 47 year old female was admitted with the chief complaints of edema and gait disturbance. Past and family histories were noncontributory. In April 1981, edema appeared in her face and legs. In June she noticed paresthesia in her legs. Edema increased gradually and she had difficulty walking. Her skin became pigmented and hairy. In October she was admitted because of polyneuropathy with increased cerebrospinal fluid protein without pleocytosis. Prednisolone was started. Walking improved slightly, but edema and paresthesia remained unchanged. Prednisolone was stopped at the end of the following March. In May 1982, she was admitted for further evaluation of edema and polyneuropathy. The patient was alert and cooperative. On standing the skin of her legs became cyanotic. There was hypertrichosis on the arms and legs. Her fingers were clubbed. A moderate swelling of the cervical lymph nodes was noted. There was mild hepatomegaly without splenomegaly. All tendon reflexes were lost. Plantar response was flexor. Muscular strength diminished mildly. She complained of paresthesia on the soles. Superficial sensation was normal. Vibratory sense decreased mildly. Cerebellar function and cranial nerves were normal. There was no sphincter disturbance. The examination of urine, stool and peripheral blood was normal.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

一种被称为伴有多神经病及各种内分泌表现的浆细胞异常增生症,或伴有多神经病、器官肿大、内分泌病、异常M蛋白及皮肤改变的浆细胞异常增生症,非常有趣,因为该综合征有多种表现,如皮肤色素沉着、多毛、多神经病、M蛋白异常、浆细胞异常增生及内分泌紊乱。本文报道了迄今尚未描述的各种内分泌异常情况。一名47岁女性因水肿和步态障碍为主诉入院。既往史和家族史均无特殊。1981年4月,她的面部和腿部出现水肿。6月,她注意到腿部有感觉异常。水肿逐渐加重,她行走困难。她的皮肤变得色素沉着且多毛。10月,她因脑脊液蛋白升高伴多神经病且无细胞增多而入院。开始使用泼尼松龙治疗。行走稍有改善,但水肿和感觉异常仍无变化。次年3月底停用泼尼松龙。1982年5月,她因水肿和多神经病进一步评估而入院。患者神志清醒,配合治疗。站立时她腿部皮肤发绀。手臂和腿部有多毛症。她的手指呈杵状。颈部淋巴结有中度肿大。有轻度肝肿大,无脾肿大。所有腱反射消失。跖反射为屈性。肌力轻度减弱。她主诉足底有感觉异常。浅感觉正常。振动觉轻度减退。小脑功能和颅神经正常。无括约肌功能障碍。尿液、粪便和外周血检查正常。(摘要截短至250字)

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