Published online Aug 26, 2021. doi: 10.12998/wjcc.v9.i24.7163
Peer-review started: January 5, 2021
First decision: April 29, 2021
Revised: May 31, 2021
Accepted: July 9, 2021
Article in press: July 9, 2021
Published online: August 26, 2021
Processing time: 230 Days and 17.3 Hours
Morbihan syndrome is a rare illness, which presents with recurrent pitting edema on the upper two thirds of the face causing facial contour deformities, and Morbihan syndrome obstructing the pupillary axis with ptosis was seldomly reported.
A 59-year-old woman presented with a 15-year history of facial swelling that progressively invaded bilaterally from the inner canthus and eyelids outwards. Imaging examination indicated that the bilateral periorbital and the left temporal soft tissues had swelling without cranium invasion. Histopathological analysis showed a large amount of lymphocyte infiltration, and immunohistochemistry showed positive expression of CD68 in clear-cut granulomas and D2-40 in the lymphatic endothelium. Finally, the clinical diagnosis of Morbihan syndrome was confirmed.
Morbihan syndrome is an infrequent and refractory disease, which is characteristic with recurrent woody facial edema on the upper two thirds of the face. Solid facial edema is persistent and non-pitting, causing facial contour deformities and even vision field impairment. The diagnosis of Morbihan syndrome depends on clinical features, imaging information, and pathology. Blepharoplasty is optional to improve the visual field for these patients with severe pupillary axis obstruction.
Core Tip: Morbihan syndrome is a rare and refractory condition that is characterized by persistent edema on the upper two thirds of the face. We report a Morbihan syndrome patient with progressive disfiguring facial masses to cause pupillary axis obstruction. The features and prognosis of this disease are carefully described to help clinical physicians know this illness.