Case Report
Copyright ©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jul 6, 2023; 11(19): 4692-4697
Published online Jul 6, 2023. doi: 10.12998/wjcc.v11.i19.4692
Morbihan disease misdiagnosed as senile blepharoptosis and successfully treated with short-term minocycline and ketotifen: A case report
Jun Na, Yan Wu
Jun Na, Yan Wu, Department of Dermatology, Peking University First Hospital, Beijing 100034, China
Author contributions: Jun Na contributed to manuscript writing and editing, and data collection; Yan Wu contributed to conceptualization and supervision; all authors have read and approved the final manuscript.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Yan Wu, MD, Chief Physician, Department of Dermatology, Peking University First Hospital, No. 8 Xishiku Street, Xicheng District, Beijing, China. 3437477565@qq.com
Received: March 13, 2023
Peer-review started: March 13, 2023
First decision: May 19, 2023
Revised: May 31, 2023
Accepted: June 14, 2023
Article in press: June 14, 2023
Published online: July 6, 2023
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.

Keywords: Morbihan disease, Ptosis, Minocycline, Mast cells, Treatment, Case report

Core Tip: Morbihan disease is a rare skin disease that is mainly characterized by nonpitting erythematous edema on the upper two-thirds of the face. We report an atypical case of progressive blepharoptosis without obvious edema, as a reminder of the existence of this uncommon condition to dermatologists, neurologists, and ophthalmologists to reduce chances of misdiagnosis. Usually long-term minocycline is used to treat Morbihan disease but has uncertain efficacy. We successfully treated our patient with a combination of minocycline and ketotifen. We also summarized previous reports on the use of minocycline in Morbihan disease and noted that mast cells might be key histological predictors of the treatment response to minocycline.