Progressive disfiguring facial masses with pupillary axis obstruction from Morbihan syndrome: A case report

Abstract

BACKGROUND 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. CASE SUMMARY 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. CONCLUSION 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.

Omalizumab in the treatment of Morbihan syndrome in an adolescent girl – case report and literature review

“…However, this problem has not been the subject of publications so far. 10,11 Our patient, though almost the youngest among those being described, presented with all typical symptoms. Usually, the diagnosis of MS is made after the exclusion of other potential causes of swelling.…”

“…When seen in the acute setting, Morbihan syndrome can be from 5 months to 5 years, 6,9,[26][27][28] reaching in extreme cases up to 15 years. 10 As the disease is extremely rare, there are no randomized trials. It is suggested that the later treatment starts, the lower the chances of a full recovery.…”

“…2,9 The disease's main symptom is a nonitchy, cohesive, erythematous swelling of the upper-and mid-face, deforming the patient's features and often restricting the visual field. 1,2,3,10 The skin lesions are usually symmetrical and exceptionally unilateral. 11,12 Pathophysiology is believed to be related to rosacea or acne vulgaris, as one of them is present in most cases of MS before the onset of the swelling.…”