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被误诊为老年性上睑下垂的莫尔比昂病经短期米诺环素和酮替芬成功治疗:一例报告

Morbihan disease misdiagnosed as senile blepharoptosis and successfully treated with short-term minocycline and ketotifen: A case report.

作者信息

Na Jun, Wu Yan

机构信息

Department of Dermatology, Peking University First Hospital, Beijing 100034, China.

出版信息

World J Clin Cases. 2023 Jul 6;11(19):4692-4697. doi: 10.12998/wjcc.v11.i19.4692.

Abstract

BACKGROUND

Morbihan disease is a rare skin condition with diagnostic and therapeutic challenges. Facial nonpitting erythematous edema is usually considered to be a characteristic manifestation and diagnostic clue for the Morbihan disease. Treatment of Morbihan disease remains a dilemma due to its long course, poor response, and high recurrence rate.

CASE SUMMARY

We report the case of a 69-year-old man with Morbihan disease. The patient presented ptosis as the first and main symptom. There was no obvious edema or other skin lesions. The patient was misdiagnosed with senile blepharoptosis based on eyelid performance and no treatment was administered to him. After continuous progressive asthenia of eye-opening and ptosis for more than one year, a skin biopsy was recommended. Histopathological analysis showed edema in the dermis, lymphatic hyperplasia and dilatation, and perivascular lymphocytic infiltration. An obvious increase in toluidine blue-stained mast cells was observed. The patient was finally diagnosed with Morbihan disease. Minocycline and ketotifen were prescribed based on the increase of mast cells in skin tissue slices. The patient experienced rapid relief seven days later and complete remission after 40 d from the commencement of the treatment.

CONCLUSION

Ptosis without obvious swelling could be the only or main clinical manifestation of Morbihan disease in rare conditions. An increase of mast cells was an important therapeutic clue to the rapid and remarkable efficiency of the combination therapy of minocycline and antihistamine.

摘要

背景

莫尔比昂病是一种罕见的皮肤病,在诊断和治疗方面存在挑战。面部非凹陷性红斑性水肿通常被认为是莫尔比昂病的特征性表现和诊断线索。由于其病程长、反应差和复发率高,莫尔比昂病的治疗仍然是一个难题。

病例摘要

我们报告了一例69岁的莫尔比昂病男性患者。患者以眼睑下垂为首发及主要症状。无明显水肿或其他皮肤病变。根据眼睑表现,患者被误诊为老年性睑下垂,未接受任何治疗。在持续进行性睁眼乏力和眼睑下垂一年多后,建议进行皮肤活检。组织病理学分析显示真皮水肿、淋巴管增生和扩张以及血管周围淋巴细胞浸润。观察到甲苯胺蓝染色的肥大细胞明显增多。患者最终被诊断为莫尔比昂病。根据皮肤组织切片中肥大细胞增多的情况,给予米诺环素和酮替芬治疗。患者在7天后迅速缓解,治疗开始40天后完全缓解。

结论

在罕见情况下,无明显肿胀的眼睑下垂可能是莫尔比昂病唯一或主要的临床表现。肥大细胞增多是米诺环素与抗组胺药联合治疗快速且显著有效的重要治疗线索。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/646a/10353504/ccee3d677860/WJCC-11-4692-g001.jpg

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