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莫尔比昂综合征

Morbihan syndrome.

作者信息

Veraldi Stefano, Persico Maria Chiara, Francia Claudia

机构信息

Department of Pathophysiology and Transplantation, University of Milan, I.R.C.C.S. Foundation, Cà Granda Ospedale Maggiore Policlinico, Milan, Italy.

出版信息

Indian Dermatol Online J. 2013 Apr;4(2):122-4. doi: 10.4103/2229-5178.110639.

Abstract

We report a case of severe Morbihan syndrome (chronic erythematous edema of the upper portion of the face) in a 60-year-old man. The syndrome was characterized clinically by erythematous edema involving the forehead, glabella, and both eyelids, because of which the patient was not able to open completely his eyes. Furthermore, erythema and telangiectasiae were visible on the nose and cheeks. Laboratory and instrumental examinations were within normal ranges or negative. Histopathological examination showed dermal edema, perivascular and periadnexal lympho-histiocytic infiltrate, and sebaceous gland hyperplasia. Oral isotretinoin was ineffective despite the relatively long duration of the therapy (26 weeks).

摘要

我们报告一例60岁男性的严重莫尔比昂综合征(面部上部慢性红斑性水肿)。该综合征临床特征为前额、眉间及双侧眼睑出现红斑性水肿,患者因此无法完全睁开眼睛。此外,鼻子和脸颊可见红斑及毛细血管扩张。实验室和器械检查结果均在正常范围内或为阴性。组织病理学检查显示真皮水肿、血管周围及附件周围淋巴细胞 - 组织细胞浸润以及皮脂腺增生。尽管治疗时间相对较长(26周),口服异维A酸仍无效。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8f5d/3673378/b4b25151511a/IDOJ-4-122-g001.jpg

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