Morbihan disease: a therapeutic challenge

Cabral, Fernanda V.; Lubbe, Lara Caroline; Nóbrega, Monisa Martins; Obadia, Daniel Lago; Souto, Roberto; Gripp, Alexandre Carlos

doi:10.1590/abd1806-4841.20174485

Cited by 18 publications

(8 citation statements)

References 7 publications

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“…Immunohistochemical studies showed D2-40 expression in the lymphatic endothelial cells, which was reported to be the typical phenotype of endothelial cells in Morbihan syndrome[ 7 ]. In addition, CD68 could also act as a histological marker for Morbihan syndrome to exclude carcinoma[ 8 ].…”

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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“…Choroba Morbihan (Morbihan disease) jest jednostką o niejasnej etiopatogenezie, najczęściej klasyfikowaną jako odmiana lub późne stadium trądziku różowatego [1,2]. Charakteryzuje się spoistym, rumieniowym, najczęściej symetrycznym obrzękiem górnej i środkowej części twarzy.…”

Section: Wprowadzenieunclassified

“…Dermatology Review/Przegląd Dermatologiczny 2020/6 introduction Morbihan disease is a condition of unclear aetiopathogenesis, most often classified as a variety or late stage of rosacea [1,2]. It is characterised by a coherent, erythematous, usually symmetrical swelling of the upper and middle part of the face.…”

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confidence: 99%

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 entity predominantly affecting Caucasian adults of both sexes in the third and fourth decade of life 1 2. The pathogenesis of the syndrome is not well elucidated 1…”

Section: Introductionmentioning

confidence: 99%

Rosacea causing unilateral Morbihan syndrome

Weeraman

Birnie

2019

BMJ Case Rep

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Morbihan syndrome is a rare entity causing woody induration of the face. There are numerous case reports of bilateral Morbihan syndrome. We present a case of a 46-year-old man with right infra-orbital cheek swelling and symptoms of rosacea who had histology consistent with granulomatous rosacea following debulking surgery. His clinical presentation and investigation findings support a diagnosis of rosacea causing unilateral Morbihan syndrome.

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Morbihan disease: a therapeutic challenge

Cited by 18 publications

References 7 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

Morbihan disease

Rosacea causing unilateral Morbihan syndrome

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