Omalizumab in the treatment of Morbihan syndrome in an adolescent girl – case report and literature review
Main Article Content
Keywords
Adolescent, anti-IgE, solid facial edema, Morbihan syndrome, omalizumab
Abstract
Morbihan syndrome (MS) is characterized by solid facial edema, usually related to rosacea or acne vulgaris. The facial edema deforms the patient’s features, can impair peripheral vision, and affects quality of life. Its pathophysiology remains unclear. The disease usually has a slow and chronic course. MS most commonly affects middle-aged Caucasian men with rosacea and is rare in people below 20 years of age. MS is a diagnosis of exclusion. There is no standard treatment for MS, though systemic isotretinoin and antihistamines are mainly used. We present the case of an adolescent girl with MS nonresponding to 19 months of isotretinoin treatment with add-on antihistamines. Therapy with monthly administration of omalizumab (anti-IgE) for 6 months was an effective therapeutic option, improving the quality of life. Our case is the second description of omalizumab use in Morbihan syndrome, the first in an adolescent.
References
2. Boparai RS, Levin AM, Lelli GJ Jr. Morbihan disease treatment: two case reports and a systematic literature review. Ophthalmic Plast Reconstr Surg. 2019 Mar/Apr;35(2):126–32. 10.1097/IOP.0000000000001229
3. Mayur O, Martinez R, McNichol MC, McGee JS. Clinical and histological features and treatment outcomes of patients with Morbihan disease: A systematic review. Arch Dermatol Res. 2023 Apr 16;315:2427–429. 10.1007/s00403-023-02621-6
4. Ramirez-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 Oct;41(10):701–10. 10.1097/DAD.0000000000001378
5. van der Linden MM, Arents BW, van Zuuren EJ. Diagnosis and treatment of Morbihan’s disease: a practical approach based on review of the Literature. J Clin Aesthet Dermatol. 2023 Oct;16(10):22–30.
6. Li ZY, Chi CC, Cai SQ. Case report: Morbihan disease treated with tofacitinib successfully. Front Immunol. 2023 Jun 5;14:1177316. 10.3389/fimmu.2023.1177316
7. Kim JH. Treatment of Morbihan disease. Arch Craniofac Surg. 2021 Jun;22(3):131–34. 10.7181/acfs.2021.00185
8. Yvon C, Mudhar HS, Fayers T, Siah WF, Malhotra R, Currie Z, et al. Morbihan Syndrome, a UK Case Series. Ophthalmic Plast Reconstr Surg. 2020 Sep–Oct;36(5):438–43. 10.1097/IOP.0000000000001589
9. Kuhn-Régnier S, Mangana J, Kerl K, Kamarachev J, French LE, Cozzio A, et al. A report of two cases of solid facial edema in acne. Dermatol Ther (Heidelb). 2017 Mar;7(1):167–174. 10.1007/s13555-017-0173-6
10. Zhang L, Yan S, Pan L, Wu SF. Progressive disfiguring facial masses with pupillary axis obstruction from Morbihan syndrome: A case report. World J Clin Cases. 2021 Aug 26;9(24):7163–68. 10.12998/wjcc.v9.i24.7163
11. Macca L, Li Pomi F, Motolese A, Broganelli P, Borgia F. Unilateral Morbihan syndrome. Dermatol Reports. 2021 Aug 5;14(2):9270. 10.4081/dr.2021.9270
12. Weeraman S, Birnie A. Rosacea causing unilateral Morbihan syndrome. BMJ Case Rep. 2019 Oct 25;12(10):e231074. 10.1136/bcr-2019-231074
13. Çinar GN, Özgül S, Nakip G, Üzelpasaci E, Baran E, Gürşen C, et al. Complex decongestive therapy in the physical therapist management of rosacea-related edema (morbus morbihan syndrome): A case report with a new approach. Phys Ther. 2021 Sep 1;101(9):pzab133.
14. Michelle Fog Andersen, Hilary J. Longhurst, Eva Rye Rasmussen, Anette Bygum. How not to be misled by disorders mimicking angioedema: A review of pseudoangioedema. Int Arch Allergy Immunol 1 June 2016; 169 (3):163–170. 10.1159/000445835
15. Kafi P, Edén I, Swartling C. Morbihan syndrome successfully treated with omalizumab. Acta Derm Venereol. 2019 Jun 1;99(7):677–78. 10.2340/00015555-3168
16. Yu L, Zhang H, Pan J, Ye L. Pediatric usage of Omalizumab: A promising one. World Allergy Organ J. 2021 Dec 11;14(12):100614. 10.1016/j.waojou.2021.100614
17. 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 Nov;33(6):764–67. 10.1111/j.1365-2230.2008.02892.x
18. García-Arpa M, Rodríguez-Vázquez M, Bellido-Pastrana D, Villasanti-Rivas N, Carmona-Rodríguez M. Persistent facial oedema and erythema in a woman, an uncommon manifestation of chronic cutaneous lupus erythematosus. Eur J Case Rep Inter Med 2020; Feb 12;7(3):1462.
19. Wohlrab J, Lueftl M, Marsch WC. Persistent erythema and oedema 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 Apr;52(4):595–602. 10.1016/j.jaad.2004.08.061
20. Veraldi S, Persico M, Francia C. Morbihan syndrome. Indian Dermatology Online Journal. 2013;Apr-Jun 4(2):122–24. 10.4103/2229-5178.110639
21. Tonkin R, Sladden C. What lies downstream? A case of superior vena cava syndrome presenting in the dermatology clinic: A case report. SAGE Open Med Case Rep. 2020 May 20;8:2050313X20. 10.1177/2050313X20919618
22. Kuraitis D, Coscarart A, Williams L, Wang A. Morbihan disease: A case report and differentiation from Melkersson-Rosenthal syndrome. Dermatol Online J. 2020 Jun 15;26(6):13030. 10.5070/D3266049317
23. Donthi D, Nenow J, Samia A, Phillips C, Papalas J, Prenshaw K. Morbihan disease: A diagnostic dilemma: two cases with successful resolution. SAGE Open Med Case Rep. 2021 Jun 11;9:2050313X211023655. 10.1177/2050313X211023655
24. Jerković Gulin S, Ljubojević Hadžavdić S. Morbihan disease–An old and rare entity still difficult to treat. Acta Dermatovenerol Croat. 2020 Aug;28(2):118–9.
25. Rizzo A, Fiorani D, Lazzeri L, Taddeucci P, Rubegni P, Flori ML, et al. Usefulness of in vivo reflectance confocal microscopy in Morbihan syndrome. Skin Res Technol. 2021 Sep;27(5):974–76. 10.1111/srt.13002
26. Guimarães MJ, Lopes AG, Vieira AP. Morbihan disease: A diagnostic and therapeutic challenge. Acta Med Port. 2023 Jun 28. 10.20344/amp.19904
27. Kim JE, Sim CY, Park AY, Hong SA, Park YL, Jang SY, et al. Case series of Morbihan disease (Extreme eyelid edema associated with rosacea): Diagnostic and Therapeutic Approaches. Ann Dermatol. 2019 Apr;31(2):196–200. 10.5021/ad.2019.31.2.196
28. Olvera-Cortés V, Pulido-Díaz N. Effective treatment of Morbihan’s disease with long-term isotretinoin: A report of three cases. J Clin Aesthet Dermatol. 2019 Jan;12(1):32–4.
29. Bagatin E, Costa CS, Rocha MADD, Picosse FR, Kamamoto CSL, Pirmez R, et al. Consensus on the use of oral isotretinoin in dermatology–Brazilian Society of Dermatology. An Bras Dermatol. 2020 Nov-Dec;95 Suppl 1(Suppl 1):19–38. 10.1016/j.abd.2020.09.001
30. Cribier B. Rosacea: Treatment targets based on new physiopathology data. Ann Dermatol Venereol. 2022 Jun;149(2):99–107. 10.1016/j.annder.2021.11.001
31. Borman P, Yaman A. Successful treatment for the different and rare cause of facial lymphedema: Morbus Morbihan disease. Int J Dermatol. 2022 Jan;61(1):103–105. 10.1111/ijd.15643
32. Anderson MS, Nadkarni A, Cardwell LA, Alinia H, Feldman SR. Spotlight on brimonidine topical gel 0.33% for facial erythema of rosacea: safety, efficacy, and patient acceptability. Patient Prefer Adherence. 2017 Jul 6;11:1143–150. 10.2147/PPA.S115708
33. Hong JH, Yi CC, Lee JW, Bae YC, Kim RS, Kim JH. Lymphaticovenular anastomosis for Morbihan disease: A case report. Arch Craniofac Surg. 2023 Jun;24(3):124–28. 10.7181/acfs.2022.01067
34. 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 Dec;32(8):941–44. 10.1080/09546634.2020.1721417
35. Skander D, Allenova A, Maurer M, Kolkhir P. Omalizumab is effective in patients with chronic spontaneous urticaria plus multiple chronic inducible urticaria. Eur Ann Allergy Clin Immunol. 2021 Mar;53(2):91–3. 10.23822/EurAnnACI.1764-1489.153
36. Zuberbier T, Maurer M. Omalizumab for the treatment of chronic urticaria. Expert Rev Clin Immunol. 2015 Feb;11(2):171–80. 10.1586/1744666X.2015.993971
37. Varghese A, Paul T, Kim H, Van Uum S, Vadas P, Azzola A. Effect of omalizumab for autoimmune progesterone dermatitis refractory to bilateral oophorectomy: A case report. Allergy Asthma Clin Immunol. 2021 Jun 13;17(1):58. 10.1186/s13223-021-00561-2
38. Sarrazin M, Jouen F, Duvert-Lehembre S. Refractory bullous pemphigoid with IgE anti-BP230 and IgG anti-p200 antibodies successfully treated with omalizumab. Ann Dermatol Venereol. 2021 Mar;148(1):60–62. 10.1016/j.annder.2020.08.053
39. Fujimoto N, Mitsuru M, Tanaka T. Successful treatment of Morbihan disease with long-term minocycline and its association with mast cell infiltration. Acta Derm Venereol. 2015 Mar;95(3):368–69. 10.2340/00015555-1954
40. Blauvelt A, Gondo GC, Bell S, et al. Psoriasis Involving special areas is associated with worse quality of life, depression, and limitations in the ability to participate in social roles and activities. Journal of Psoriasis and Psoriatic Arthritis. 2023;8(3):100–106. 10.1177/24755303231160683