Novel prognostic factors and combination therapy outcomes in Morbihan disease: insights from an Asian population

Jungyul Park; Moon-Bum Kim; Hee-Young Choi; Suk-Woo Yang

doi:10.1186/s12886-024-03758-2

Novel prognostic factors and combination therapy outcomes in Morbihan disease: insights from an Asian population

BMC Ophthalmol. 2024 Nov 13;24(1):496. doi: 10.1186/s12886-024-03758-2.

Authors

Jungyul Park¹, Moon-Bum Kim², Hee-Young Choi³, Suk-Woo Yang⁴

Affiliations

¹ Department of Ophthalmology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, #222 Banpo-daero, Seocho-gu, Seoul, 06591, Korea.
² Department of Dermatology, Pusan National University College of Medicine, Busan, Korea.
³ Department of Ophthalmology, Pusan National University College of Medicine, Busan, Korea.
⁴ Department of Ophthalmology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, #222 Banpo-daero, Seocho-gu, Seoul, 06591, Korea. yswoph@catholic.ac.kr.

PMID: 39538162
DOI: 10.1186/s12886-024-03758-2

Abstract

Purpose: This study aimed to identify novel prognostic factors for Morbihan disease (MD) treatment outcomes and evaluate the efficacy of combination therapy in an Asian population, addressing the gaps in current understanding of this rare condition.

Methods: We conducted a retrospective analysis of MD patients diagnosed and treated at a tertiary hospital between 2017 and 2023. Patients received combinations of oral medications (tetracycline, isotretinoin, corticosteroids), topical treatments (tacrolimus, ivermectin), and intralesional steroid injections. Treatment response (TR) was defined as complete symptom remission, while partial remission or recurrence was considered a poor response (PR). Clinical, histological, and biochemical parameters were analyzed to identify prognostic factors.

Results: The study included 24 patients (18 men; mean age 61.3 years). Ten patients (41.7%) achieved TR, while 14 (58.3%) showed PR. Significant prognostic factors for TR included shorter symptom duration (≤ 3 months, p = 0.016), lower LDL cholesterol levels (≤ 89 mg/dL, p = 0.046), combination treatment with oral and topical medications (p = 0.033 at 6 months), and partial response at 1 month (p = 0.017). GLMM analysis identified the number of visits (p < 0.001), symptom duration (p = 0.020), and dyslipidemia (p = 0.006) as significant prognostic factors. Histologically, perivascular and perifollicular lymphocytic infiltration were the most common findings (83.3%). Notably, 50% of patients were ANA-positive, challenging previous diagnostic criteria.

Conclusion: This study identifies novel prognostic factors for favorable outcomes in MD, including early intervention and lipid management. Combination therapy, particularly with tacrolimus ointment, shows promise in improving treatment responses. These findings suggest a potential link between lipid metabolism and MD pathophysiology, opening new avenues for targeted therapies.

Keywords: Chronic lymphedema; Eyelid lymphedema; Morbihan disease; Morbihan syndrome; Rosaceous lymphedema.