Published online Jul 6, 2023. doi: 10.12998/wjcc.v11.i19.4692
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
Processing time: 108 Days and 23.3 Hours
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.
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 lym
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.
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.