Morbihan Syndrome, a UK Case Series

Yvon, Camille; Mudhar, Hardeep Singh; Fayers, Tessa; Siah, We Fong; Malhotra, Raman; Currie, Zanna; Tan, Jennifer; Rajak, Saul

doi:10.1097/iop.0000000000001589

Cited by 16 publications

(25 citation statements)

References 26 publications

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“…Morbihan syndrome is so uncommon that it is only reported in case studies [6,[11][12][13]. No previous case reports link the syndrome to TN or show improvement in symptoms following treatment of TN with SRS.…”

Section: Discussionmentioning

confidence: 99%

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Treatment of Trigeminal Neuralgia With Stereotactic Radiosurgery Improved Symptoms of Morbihan Syndrome

Burgess¹,

Mula‐Hussain²,

Malone³

2021

Cureus

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Trigeminal neuralgia (TN) is a neuropathic pain disorder characterized by paroxysmal pain in the maxillary and mandibular regions of the face. Morbihan syndrome is a disease that classically presents with dermatologic findings, including progressive facial edema and erythema. There are no previous reports of the onset of trigeminal neuralgia with Morbihan syndrome or previous reports describing improvement in symptoms of Morbihan syndrome with treatment of trigeminal neuralgia. We describe the case of a 62-yearold female who presented with trigeminal neuralgia and shortly thereafter developed significant facial edema and was diagnosed with Morbihan syndrome. The neuralgia was refractory to medical management and was effectively treated with stereotactic radiosurgery (SRS). This coincided with an improvement in her Morbihan syndrome that is now controlled following stereotactic radiosurgery and continued lymphatic massage.

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Section: Discussionmentioning

confidence: 99%

“…Patients typically present with significant swelling of the upper two-thirds of their faces. The edema can be so severe that it can result in disfigurement and visual field deficits [ 6 ]. Other common features include facial flushing, telangiectasia, papules, nodules, and granulomas [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

Treatment of Trigeminal Neuralgia With Stereotactic Radiosurgery Improved Symptoms of Morbihan Syndrome

Burgess¹,

Mula‐Hussain²,

Malone³

2021

Cureus

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“…Because of the rarity of Morbihan disease, there are no prospective studies of its treatment. Therapeutic options include the following: oral isotretinoin, antihistamines (including ketotifen), systemic antibiotic therapy (tetracyclines, metronidazole), systemic corticotherapy, thalidomide, clofazimine, triamcinolone injections, and surgical methods (blepharoplasty, debulking surgery) [9,12,13]. The literature is dominated by reports of moderate effectiveness of isotretinoin [14].…”

Section: Discussionmentioning

confidence: 99%

“…Do opcji terapeutycznych należą: doustna izotretynoina, leki antyhistaminowe (m.in. ketotifen), ogólna antybiotykoterapia (tetracykliny), metronidazol, ogólna glikokortykosteroidoterapia, talidomid, klofazymina, iniekcje z triamcynolonu oraz metody chirurgiczne (blefaroplastyka, chirurgia odciążająca) [9,12,13]. W literaturze dominują doniesienia o umiarkowanej skuteczności izotretynoiny [14].…”

Section: Discussionunclassified

Morbihan disease

Dominiak¹,

Oszukowska²,

Lesiak³

2020

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Introduction. Morbihan disease is a condition of unclear aetiology characterised by solid facial swelling, usually in the upper and middle parts of the face. The disease may develop as a complication of rosacea, with intense erythema and telangiectasias. It mainly affects middleaged men. Objective. To present a case of a patient with rare Morbihan disease, who was treated with isotretinoin and ketotifen. Case report. A 45-year-old patient came to the Department of Dermatology due to an erythematous facial swelling lasting about one year. Laboratory tests showed elevated liver parameters with no other abnormalities. Because of a significant exacerbation of skin lesions, treatment with isotretinoin and ketotifen was initiated. Improvement was observed after 11 months of treatment. Conclusions. Differential diagnosis of Morbihan disease should take into consideration oedema in the course of systemic diseases and other causes of localised lymphoedema. Histopathological examination is recommended for diagnosis. streszczenie Wprowadzenie. Choroba Morbihan jest jednostką o niejasnej etiologii, którą charakteryzuje lity obrzęk twarzy, zazwyczaj górnej i środkowej części. Choroba może rozwijać się jako powikłanie trądziku różowatego z nasilonym rumieniem i teleangiektazjami. Dotyka głównie mężczyzn w średnim wieku. Cel pracy. Przedstawienie przypadku pacjenta z rzadko występującą chorobą Morbihan, która była leczona izotretynoiną i ketotifenem. Opis przypadku. Pacjent, lat 45, zgłosił się do Kliniki Dermatologii z powodu rumieniowego obrzęku twarzy trwającego od około roku. Badania laboratoryjne wykazały podwyższone parametry wątrobowe, bez innych odchyleń. Ze względu na znaczne nasilenie zmian skórnych wdrożono leczenie izotretynoiną z ketotifenem. Obserwowano poprawę po 11 miesiącach leczenia. Wnioski. Diagnostyka różnicowa powinna uwzględniać obrzęki w przebiegu chorób ogólnoustrojowych oraz inne przyczyny zlokalizowanego obrzęku limfatycznego. W celu potwierdzenia rozpoznania zaleca się badanie histopatologiczne.

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“…Morbihan syndrome, first reported in 1957 by Robert Degos, is a rare, refractory, but striking disease[ 1 ]. Typically, Morbihan syndrome presents with recurrent pitting edema on the upper two thirds of the face.…”

Section: Introductionmentioning

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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Morbihan Syndrome, a UK Case Series

Cited by 16 publications

References 26 publications

Treatment of Trigeminal Neuralgia With Stereotactic Radiosurgery Improved Symptoms of Morbihan Syndrome

Treatment of Trigeminal Neuralgia With Stereotactic Radiosurgery Improved Symptoms of Morbihan Syndrome

Morbihan disease

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

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