Extreme Eyelid Lymphedema Associated With Rosacea (Morbihan Disease): Case Series, Literature Review, and Therapeutic Considerations

Carruth, Bryant P.; Meyer, Dale R.; Wladis, Edward J.; Bradley, Elizabeth; Al‐Rohil, Rami N.; Jones, David; Bartley, George B.

doi:10.1097/iop.0000000000000581

Cited by 26 publications

(26 citation statements)

References 15 publications

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“…The pathogenic mechanisms of Morbihan syndrome are unclear, and some dermatologists recognized it as one of complications of rosacea[ 9 ]. However, there are also some reported cases of Morbihan syndrome that show no clinical manifestations of rosacea, which are similar to our case[ 10 ].…”

Section: Discussionmentioning

confidence: 99%

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

Zhang¹,

Yan²,

Pan³

et al. 2021

WJCC

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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.

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Section: Discussionmentioning

confidence: 99%

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

Zhang¹,

Yan²,

Pan³

et al. 2021

WJCC

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“…Surgery is reserved for failure of primary medical therapy without significant improvement in symptoms of periocular involvement such as visual obstruction or ectropion. 5 – 7 , 9 Most reported cases have muscular and septal involvement with fibrosis and edema. 5 , 9 For upper eyelids, most surgeons report using the standard blepharoplasty incision.…”

Section: Discussionmentioning

confidence: 99%

“… 5 , 9 For upper eyelids, most surgeons report using the standard blepharoplasty incision. 5 , 7 For lower lids, most utilize a subciliary incision and excise involved portions of the orbicularis oculi muscle and orbital septum. 5 , 7 , 9 Our patient had significant periocular involvement with visual obstruction and involvement of the orbicularis oculi muscle and orbital septum, which were resected.…”

Section: Discussionmentioning

confidence: 99%

“… 5 , 7 For lower lids, most utilize a subciliary incision and excise involved portions of the orbicularis oculi muscle and orbital septum. 5 , 7 , 9 Our patient had significant periocular involvement with visual obstruction and involvement of the orbicularis oculi muscle and orbital septum, which were resected. In one case report, surgeons utilized CO 2 laser cautery during surgery with the debulking via blepharoplasty technique.…”

Section: Discussionmentioning

confidence: 99%

“… 1 Morbihan’s disease, especially cases resistant to medical treatment with periocular involvement, can lead to cosmetic disfigurement and visual obstruction, for which surgical intervention is warranted. 1 , 5 – 7 …”

mentioning

confidence: 99%

See 2 more Smart Citations

A Navajo Patient with Morbihan’s Disease: Insight into Oculoplastic Treatment of a Rare Disease

Pflibsen

Howarth

Rochin

et al. 2020

Plastic and Reconstructive Surgery - Global Open

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Summary: Morbihan’s disease is classically defined as primarily a Caucasian disease of chronic, recurrent erythema and nonpitting edema of the middle and upper thirds of the face. The disease is often difficult to diagnose and challenging to treat. Medical management is the primary treatment modality; however, if there is progression of the disease that causes visual impairment, surgical debulking is often required. We present the first reported case of Morbihan’s disease in a Navajo patient in conjunction with a review of the literature on surgical management of this challenging disease.

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Successful treatment of a Morbihan's disease patient after a therapeutic challenge: A case report and comprehensive literature review

Bazargan,

Dehnavi,

Dehghani

et al. 2024

J of Gen and Family Med

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Morbihan's disease is a rare condition characterized by chronic facial edema. While its exact cause is unknown, it is thought to involve local cutaneous vascularization and lymphatic drainage imbalance. Traditional treatment options are often ineffective, and no established efficient treatment exists. We present a case study of a 17‐year‐old male with Morbihan's syndrome who showed resistance to traditional treatments but responded well to a combination of cromolyn sodium nasal spray and oral montelukast after histopathology revealed hyperplasia of plasma cells and mast cells. This combination has not been used before for Morbihan's syndrome. Our review of the literature also provides insight for clinicians seeking to manage this condition.

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Extreme Eyelid Lymphedema Associated With Rosacea (Morbihan Disease): Case Series, Literature Review, and Therapeutic Considerations

Cited by 26 publications

References 15 publications

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

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

A Navajo Patient with Morbihan’s Disease: Insight into Oculoplastic Treatment of a Rare Disease

Successful treatment of a Morbihan's disease patient after a therapeutic challenge: A case report and comprehensive literature review

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