medigraphic.com
ISSN 1405-1710 (Print)

2023, Number 1

Next >>

Rev Cent Dermatol Pascua 2023; 32 (1)

Morbihan disease. Clinicopathological aspects in 15 patients treated at the Centro Dermatológico ''Dr. Ladislao de la Pascua''

Navarrete-Franco G, Jurado-Santa CF, Guarneros-Campos A, Ramos-Garibay JA, González-González M, Baldassarri-Ortego LF, Marín-Rivera D

Language: Spanish
References: 18
Page: 5-10
PDF size: 469.60 Kb.


ABSTRACT

Objective: to know the epidemiology and clinical-pathological characteristics of Morbihan's disease at the Centro Dermatológico "Dr. Ladislao de la Pascua" (CDP) and a review of the literature. Material and methods: retrospective and observational study, the files of the dermatopathology service of the CDP were reviewed in a period of 32 years (January 1989 to December 2021), patients with clinical-pathological correlation corresponded to Morbihan's disease were selected. Results: 15 patients diagnosed with Morbihan's disease were identified, 13 of which (86.6%) were male. Age ranged from 19 to 65, with a mean of 35 years. In all cases, the face was the most affected topography, 12 bilaterally and symmetrically and three in which it was unilateral. Morphologically, erythema and edema predominated. The evolution was from three months to 10 years and asymptomatic. Histopathologically highlighted a granulomatous process. Conclusions: this entity is rare, and the final diagnosis is the result of a clinical-pathological correlation.


REFERENCES

  1. Saúl A, Novales J. Edema eritematoso crónico facial superior. Rev Leprol Fontilles. 1982; 13: 643-650.

  2. Boparai RS, Levin AM, Lelli GJ Jr. Morbihan disease treatment: two case reports and a systematic literature review. Ophthalmic Plast Reconstr Surg. 2019; 35: 126-132.

  3. Kafi P, Edén I, Swartling C. Morbihan syndrome successfully treated with omalizumab. Acta Derm Venereol. 2019; 99: 677-678.

  4. Aboutaam A, Hali F, Baline K, Regragui M, Marnissi F, Chiheb S. Morbihan disease: treatment difficulties and diagnosis: a case report. Pan Afr Med J. 2018; 30: 226.

  5. Carruth BP, Meyer DR, Wladis EJ, Bradley EA, Al-Rohil R, Jones DM et al. Extreme eyelid lymphedema associated with rosacea (Morbihan disease): case series, literature review, and therapeutic considerations. Ophthalmic Plast Reconstr Surg. 2017; 33: S34-S38.

  6. Belousova IE, Kastnerova L, Khairutdinov VR, Kazakov DV. Unilateral periocular intralymphatic histiocytosis, associated with rosacea (Morbihan disease). Am J Dermatopathol. 2020; 42: 452-454.

  7. Wohlrab J, Lueftl M, Marsch WC. Persistent erythema and edema of the midthird and upper aspect of the face (morbus Morbihan): evidence of hidden immunologic contact urticaria and impaired lymphatic drainage. J Am Acad Dermatol. 2005; 52: 595-602.

  8. Smith LA, Cohen DE. Successful long-term use of oral isotretinoin for the management of Morbihan disease: a case series report and review of the literature. Arch Dermatol. 2012; 148: 1395-1398.

  9. Veraldi S, Persico MC, Francia C. Morbihan syndrome. Indian Dermatol Online J. 2013; 4: 122-124.

  10. Nagasaka T, Koyama T, Matsumura K, Chen KR. Persistent lymphoedema in Morbihan disease: formation of perilymphatic epithelioid cell granulomas as a possible pathogenesis. Clin Exp Dermatol. 2008; 33: 764-767.

  11. Jansen T, Regele D, Schirren CG, Trautner B, Plewig G. Persistierendes erythem und odem des gesichts bei rosazea und lymphgefassdysplasie. Hautarzt. 1998; 49: 932-935.

  12. de Vasconcelos RCF, Eid NT, Eid RT, Moriya FS, Braga BB, Michalany AO. Morbihan syndrome: a case report and literature review. An Bras Dermatol. 2016; 91: 157-159.

  13. Okubo A, Takahashi K, Akasaka T, Amano H. Four cases of Morbihan disease successfully treated with doxycycline. J Dermatol. 2017; 44: 713-716.

  14. Tsiogka A, Koller J. Efficacy of long-term intralesional triamcinolone in Morbihan's disease and its possible association with mast cell infiltration. Dermatol Ther. 2018; 31: e12609.

  15. Ramírez-Bellver JL, Pérez-González YC, Chen KR, Díaz-Recuero JL, Requena L, Carlson JA et al. Clinicopathological and immunohistochemical study of 14 cases of Morbihan disease: an insight into its pathogenesis. Am J Dermatopathol. 2019; 41: 701-710.

  16. Cabral F, Lubbe LC, Nóbrega MM, Obadia DL, Souto R, Gripp AC. Morbihan disease: a therapeutic challenge. An Bras Dermatol. 2017; 92: 847-850.

  17. Kutlay S, Ozdemir EC, Pala Z, Ozen S, Sanli H. Complete decongestive therapy is an option for the treatment of rosacea lymphedema (Morbihan disease): two cases. Phys Ther. 2019; 99: 406-410.

  18. Welsch K, Schaller M. Combination of ultra-low-dose isotretinoin and antihistamines in treating Morbihan disease - a new long-term approach with excellent results and a minimum of side effects. J Dermatolog Treat. 2021; 32: 941-944. doi: 10.1080/09546634.2020.1721417.




2020     |     www.medigraphic.com