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Cha HG, Hyun DY, Park ES, Choi CY, Nam SM. Successful Treatment of Primary Eyelid Lymphedema by Periorbital Lymphovenous Anastomosis: A Case Report. Arch Plast Surg 2025; 52:110-115. [PMID: 40083614 PMCID: PMC11896721 DOI: 10.1055/s-0044-1792168] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/27/2024] [Accepted: 10/01/2024] [Indexed: 03/16/2025] Open
Abstract
Eyelid lymphedema is a rare condition that presents as persistent swelling and non-pitting edema of the eyelids. Treatment options for this disease are limited, including surgical debulking and medications, which do not achieve complete resolution. Few studies have demonstrated the use of lymphovenous anastomosis (LVA) in the preauricular area for eyelid lymphedema treatment. In this report, we demonstrate the successful treatment of primary eyelid lymphedema by performing multiple LVAs in the periorbital region, where dermal backflow was visualized using indocyanine green lymphography. A total of four LVAs were performed through two separate incisions at the lateral canthal area and lateral eyebrow in a patient with unilateral upper eyelid lymphedema that resulted in significant improvement without recurrence.
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Affiliation(s)
- Han Gyu Cha
- Department of Plastic and Reconstructive Surgery, Soonchunhyang University Bucheon Hospital, Soonchunhyang University College of Medicine, Bucheon, Korea
| | - Dong Yun Hyun
- Department of Plastic and Reconstructive Surgery, Soonchunhyang University Bucheon Hospital, Soonchunhyang University College of Medicine, Bucheon, Korea
| | - Eun Soo Park
- Department of Plastic and Reconstructive Surgery, Soonchunhyang University Bucheon Hospital, Soonchunhyang University College of Medicine, Bucheon, Korea
| | - Chang Yong Choi
- Department of Plastic and Reconstructive Surgery, Soonchunhyang University Bucheon Hospital, Soonchunhyang University College of Medicine, Bucheon, Korea
| | - Seung Min Nam
- Department of Plastic and Reconstructive Surgery, Soonchunhyang University Bucheon Hospital, Soonchunhyang University College of Medicine, Bucheon, Korea
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Park J, Kim MB, Choi HY, Yang SW. Novel prognostic factors and combination therapy outcomes in Morbihan disease: insights from an Asian population. BMC Ophthalmol 2024; 24:496. [PMID: 39538162 PMCID: PMC11559205 DOI: 10.1186/s12886-024-03758-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/01/2024] [Accepted: 11/05/2024] [Indexed: 11/16/2024] Open
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.
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Affiliation(s)
- Jungyul Park
- Department of Ophthalmology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, #222 Banpo-daero, Seocho-gu, Seoul, 06591, Korea
| | - Moon-Bum Kim
- Department of Dermatology, Pusan National University College of Medicine, Busan, Korea
| | - Hee-Young Choi
- Department of Ophthalmology, Pusan National University College of Medicine, Busan, Korea
| | - Suk-Woo Yang
- Department of Ophthalmology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, #222 Banpo-daero, Seocho-gu, Seoul, 06591, Korea.
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Bazargan AS, Dehnavi AZ, Dehghani A, Hesari KK, Jafari P, Goodarzi A. Successful treatment of a Morbihan's disease patient after a therapeutic challenge: A case report and comprehensive literature review. J Gen Fam Med 2024; 25:232-236. [PMID: 38966648 PMCID: PMC11221058 DOI: 10.1002/jgf2.690] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/28/2023] [Revised: 03/13/2024] [Accepted: 03/21/2024] [Indexed: 07/06/2024] Open
Abstract
Morbihan's disease is a rare condition characterized by chronic facial edema. While its exact cause is unknown, it is thought to involve local cutaneous vascularization and lymphatic drainage imbalance. Traditional treatment options are often ineffective, and no established efficient treatment exists. We present a case study of a 17-year-old male with Morbihan's syndrome who showed resistance to traditional treatments but responded well to a combination of cromolyn sodium nasal spray and oral montelukast after histopathology revealed hyperplasia of plasma cells and mast cells. This combination has not been used before for Morbihan's syndrome. Our review of the literature also provides insight for clinicians seeking to manage this condition.
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Affiliation(s)
- Afsaneh Sadeghzadeh Bazargan
- Department of Dermatology, Rasool Akram Medical Complex Clinical Research Development Center (RCRDC), School of MedicineIran University of Medical Sciences (IUMS)TehranIran
| | | | - Abbas Dehghani
- Department of Dermatology, Rasool Akram Medical Complex Clinical Research Development Center (RCRDC), School of MedicineIran University of Medical Sciences (IUMS)TehranIran
| | - Kambiz Kamyab Hesari
- Department of Dermatopathology, Razi HospitalTehran University of Medical SciencesTehranIran
| | - Paria Jafari
- Department of Dermatology, Rasool Akram Medical Complex Clinical Research Development Center (RCRDC), School of MedicineIran University of Medical Sciences (IUMS)TehranIran
| | - Azadeh Goodarzi
- Department of Dermatology, Rasool Akram Medical Complex Clinical Research Development Center (RCRDC), School of MedicineIran University of Medical Sciences (IUMS)TehranIran
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Mazurek-Durlak Z, Mularczyk K, Kwinta P, Lis G, Cichocka-Jarosz E. Omalizumab in the treatment of Morbihan syndrome in an adolescent girl - case report and literature review. Allergol Immunopathol (Madr) 2024; 52:23-31. [PMID: 38459887 DOI: 10.15586/aei.v52i2.978] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/19/2023] [Accepted: 12/15/2023] [Indexed: 03/11/2024]
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.
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Affiliation(s)
- Zofia Mazurek-Durlak
- Faculty of Medicine, Department of Pediatrics, Jagiellonian University Medical College, 265, Wielicka Str. 30-663 Krakow, Poland;
| | - Katarzyna Mularczyk
- Faculty of Medicine, Department of Pediatrics, Jagiellonian University Medical College, 265, Wielicka Str. 30-663 Krakow, Poland
| | - Przemko Kwinta
- Faculty of Medicine, Department of Pediatrics, Jagiellonian University Medical College, 265, Wielicka Str. 30-663 Krakow, Poland
| | - Grzegorz Lis
- Faculty of Medicine, Department of Pediatrics, Jagiellonian University Medical College, 265, Wielicka Str. 30-663 Krakow, Poland
| | - Ewa Cichocka-Jarosz
- Faculty of Medicine, Department of Pediatrics, Jagiellonian University Medical College, 265, Wielicka Str. 30-663 Krakow, Poland
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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. THE JOURNAL OF CLINICAL AND AESTHETIC DERMATOLOGY 2023; 16:22-30. [PMID: 37915334 PMCID: PMC10617896] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Subscribe] [Scholar Register] [Indexed: 11/03/2023]
Abstract
Background Morbihan's disease (MD), also known as solid persistent facial edema, solid facial lymphedema or rosacea lymphedema, is a rare condition. Objective Despite existing case reports and literature reviews, clinical guidance for diagnosis and management is lacking. This review aims to provide comprehensive information on the etiology, differential diagnoses, diagnostics, and management of MD. Methods PubMed was searched up to April 2023 for relevant studies on MD with no language restriction. Furthermore, references were checked of found reports. Results A comprehensive overview of the literature and clinical guidance for MD. We found 95 studies involving 166 patients (118 male, 46 female and 2 gender unreported) evaluating management options, categorized into: isotretinoin (16 studies), isotretinoin plus antihistamines (8), isotretinoin plus corticosteroids (8), antibiotics (13), antibiotics plus corticosteroids (7), surgical debulking (10) and miscellaneous/combination treatments (43). Some studies contributed to two categories. Treatment with isotretinoin as monotherapy or combined with antihistamines, doxycycline or minocycline as well as surgical procedures demonstrated mostly satisfactory results, although recurrences were common. Longer treatment duration, of at least 6 to 12 months, is recommended for pharmacological treatments. Adding systemic or intralesional corticosteroids to previous treatments may be beneficial. Manual lymph drainage seems to contribute to satisfying result. Limitations This is not a systematic review and randomized controlled trials are lacking. Conclusion Diagnosis of MD is based on specific clinical features and excluding diseases with similar appearance. Prolonged treatment is often necessary to obtain satisfactory results, which might be limited to a partial and/or temporary response.
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Affiliation(s)
- Mireille Md van der Linden
- Dr. van der Linden is with the Department of Dermatology at Amsterdam University Medical Center in Amsterdam, Netherlands
| | - Bernd Wm Arents
- Mr. Arents is with Skin Netherlands in 's-Hertogenbosch, Netherlands
| | - Esther J van Zuuren
- Dr. van Zuuren is with the Department of Dermatology at Leiden University Medical Center in Leiden, Netherlands
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Li M, Tao M, Zhang Y, Pan R, Gu D, Xu Y. Effect of High-Intensity Macro-Focused Ultrasound on a Case of Morbihan Disease. Clin Cosmet Investig Dermatol 2023; 16:1949-1954. [PMID: 37519942 PMCID: PMC10386836 DOI: 10.2147/ccid.s417070] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/14/2023] [Accepted: 07/18/2023] [Indexed: 08/01/2023]
Abstract
Morbihan disease is a rare entity involving the upper two-thirds of the face and characterized by chronic erythematous edema, which is recalcitrant to the traditional therapy of rosacea. We report a case of Morbihan disease effectively treated with macro-focused high-intensity focus ultrasound (MF-HIFU). After MF-HIFU treatment, an obvious reduction in swelling was observed with decreased erythema and hyperalgesia. In addition, in-vitro experiments were conducted to measure the actual temperature of the skin tissue under the epidermis. The results of the in-vitro experiments showed that the temperature plateau in the skin sample was reached at approximately 42°C after 5-min treatment or longer. MF-HIFU might be a promising energy-based therapy for Morbihan disease.
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Affiliation(s)
- Min Li
- Department of Dermatology, the First Affiliated Hospital of Nanjing Medical University (Jiangsu Province Hospital), Nanjing, 210029, People’s Republic of China
| | - Meng Tao
- Department of Dermatology, the First Affiliated Hospital of Nanjing Medical University (Jiangsu Province Hospital), Nanjing, 210029, People’s Republic of China
| | - Yue Zhang
- Department of Dermatology, the First Affiliated Hospital of Nanjing Medical University (Jiangsu Province Hospital), Nanjing, 210029, People’s Republic of China
| | - Ruoxin Pan
- Department of Dermatology, the First Affiliated Hospital of Nanjing Medical University (Jiangsu Province Hospital), Nanjing, 210029, People’s Republic of China
| | - Duoduo Gu
- Department of Dermatology, the First Affiliated Hospital of Nanjing Medical University (Jiangsu Province Hospital), Nanjing, 210029, People’s Republic of China
| | - Yang Xu
- Department of Dermatology, the First Affiliated Hospital of Nanjing Medical University (Jiangsu Province Hospital), Nanjing, 210029, People’s Republic of China
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Na J, Wu Y. Morbihan disease misdiagnosed as senile blepharoptosis and successfully treated with short-term minocycline and ketotifen: A case report. World J Clin Cases 2023; 11:4692-4697. [PMID: 37469741 PMCID: PMC10353504 DOI: 10.12998/wjcc.v11.i19.4692] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/13/2023] [Revised: 05/31/2023] [Accepted: 06/14/2023] [Indexed: 06/30/2023] Open
Abstract
BACKGROUND Morbihan disease is a rare skin condition with diagnostic and therapeutic challenges. Facial nonpitting erythematous edema is usually considered to be a characteristic manifestation and diagnostic clue for the Morbihan disease. Treatment of Morbihan disease remains a dilemma due to its long course, poor response, and high recurrence rate.
CASE SUMMARY We report the case of a 69-year-old man with Morbihan disease. The patient presented ptosis as the first and main symptom. There was no obvious edema or other skin lesions. The patient was misdiagnosed with senile blepharoptosis based on eyelid performance and no treatment was administered to him. After continuous progressive asthenia of eye-opening and ptosis for more than one year, a skin biopsy was recommended. Histopathological analysis showed edema in the dermis, lymphatic hyperplasia and dilatation, and perivascular lymphocytic infiltration. An obvious increase in toluidine blue-stained mast cells was observed. The patient was finally diagnosed with Morbihan disease. Minocycline and ketotifen were prescribed based on the increase of mast cells in skin tissue slices. The patient experienced rapid relief seven days later and complete remission after 40 d from the commencement of the treatment.
CONCLUSION Ptosis without obvious swelling could be the only or main clinical manifestation of Morbihan disease in rare conditions. An increase of mast cells was an important therapeutic clue to the rapid and remarkable efficiency of the combination therapy of minocycline and antihistamine.
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Affiliation(s)
- Jun Na
- Department of Dermatology, Peking University First Hospital, Beijing 100034, China
| | - Yan Wu
- Department of Dermatology, Peking University First Hospital, Beijing 100034, China
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