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[梅尔克森-罗森塔尔综合征的慢性病程及诊断疑问。5例发展方式分析]

[Chronicity and diagnostic doubts of Melkersson-Rosenthal syndrome. Analysis of developing ways in 5 cases].

作者信息

Stosiek N, Birolleau S, Capesius C, Hornstein O P

机构信息

Clinique Dermatologique de l'Université d'Erlangen-Nuremberg.

出版信息

Ann Dermatol Venereol. 1992;119(9):635-8.

PMID:1285587
Abstract

In order to demonstrate the variability of the muco-cutaneous and neurovegetative signs and symptoms of Melkersson-Rosenthal syndrome (MRS), we report the cases of five out of 80 patients suffering from this complex oro-facial syndrome. In the majority of our patients the major symptoms, and in particular recurrent labial or intraoral swellings and/or facial palsy, did not occur simultaneously, which delayed the definite diagnosis of either complete or incomplete type of MRS. However, more than 80 p. 100 of our patients complained of various relapsing cranio-facial neurovegetative troubles that constitute the so-called minor symptoms of MRS, whereas a history or sequelae of facial palsy were found in only 35 p. 100 of our patients followed up for years or decades. In patients with solitary facial palsy or oro-facial oedemas as the initial symptom of putative MRS, the uncovering of concomitant minor symptoms proves to be helpful for substantiating the recognition of incomplete MRS. Thus, in questionable cases of MRS attention should be paid to seemingly incidental disturbances of the cranio-facial neurovegetative system (e. g., relapsing disorders of lacrimation, sweating, migraine-like headache) forming minor but characteristic hints to incomplete or unusual cases of MRS.

摘要

为了证明梅尔克森 - 罗森塔尔综合征(MRS)黏膜皮肤和神经植物体征及症状的变异性,我们报告了80例患有这种复杂口腔面部综合征患者中的5例病例。在我们的大多数患者中,主要症状,特别是复发性唇部或口腔内肿胀和/或面瘫,并非同时出现,这延迟了对完全型或不完全型MRS的确切诊断。然而,超过80%的患者抱怨有各种复发性颅面部神经植物功能障碍,这些构成了所谓的MRS轻微症状,而在我们随访多年或数十年的患者中,仅35%的患者有面瘫病史或后遗症。对于以孤立性面瘫或口腔面部水肿作为疑似MRS初始症状的患者,发现伴随的轻微症状有助于证实对不完全型MRS的诊断。因此,在MRS可疑病例中,应注意颅面部神经植物系统看似偶然的紊乱(例如,复发性流泪、出汗、偏头痛样头痛),这些是不完全型或不典型MRS病例的轻微但特征性线索。

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