Clinicopathological and Immunohistochemical Study of 14 Cases of Morbihan Disease: An Insight Into Its Pathogenesis

Abstract

Introduction: Morbihan disease (MORD) is rare with only 45 clear-cut cases previously reported. Histopathologic findings are supposed to be nonspecific. We report 14 patients and review the previous cases. Objectives: To characterize the clinicopathologic findings, outcomes, and immunophenotype of MORD. Material and Methods: Inclusion criteria were a clinical picture of persistent, nonpitting edema affecting the mid and or upper third of the face and histopathological findings fitting previous reports and exclusion of other entities. Results: The majority of our patients were males (71.5%) with a male/female ratio of 10/4. The mean age when diagnosed was 58.8 years. Eyelids and forehead were the most frequently involved areas. Two of the patients presented previous rosacea. Most constant histopathological findings were lymphatic vessel dilatations in the upper dermis and the presence of mast cells (7.5 in 10 high-power field as a mean). Mild edema was also present in most of the cases. Granulomas were found in 7 of the cases, and immunostaining with CD68 and CD14 only revealed an additional case. Conclusions: MORD occurs more in middle-aged males, not associated with rosacea and mostly affects eyelids and forehead. Granulomas are not mandatory for the diagnosis. Histopathology of MORD fits within the spectrum of localized lymphedema.

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

“…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 ].…”

Morbihan disease

“…Pathogenesis of solid facial oedema remains unclear, although lymphatic dysfunction is likely to play an important role [8]. Ramirez-Bellver et al [9] showed that in cases of Morbihan disease, histopathological examination may reveal oedema of the dermis, presence of dilated lymphatic vessels in the upper layers of the dermis expressing D2-40 and CD31 antigens, presence of mast cells, and, less frequently, granulomatous lymphangitis. These features correspond to localised lymphoedema; therefore, it is necessary to differentiate it from other diseases associated with lymphoedema, including Melkersson-Rosenthal syndrome [9].…”

“…Ramirez-Bellver i wsp. [9] wykazali, że w przypadkach choroby Morbihan badanie histopatologiczne może ujawnić obrzęk skóry właściwej, obecność poszerzonych naczyń limfatycznych w górnych warstwach skóry właściwej wykazujących ekspresję antygenów D2-40 i CD31, mastocytów i rzadziej -ziarniniakowe zapalenie naczyń chłonnych. Cechy te odpowiadają zlokalizowanemu obrzękowi chłonnemu, dlatego konieczne jest różnicowanie z innymi chorobami przebiegającymi z obrzękiem chłonnym, w tym z zespołem Melkerssona-Rosenthala [9].…”

“…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].…”

“…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].…”