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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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Chen L, Duan Y, Zhao L, Xu M, Liu Y, Zhai X. Case report: A therapeutic attempt to treat Morbihan disease with Baricitinib. Heliyon 2024; 10:e35547. [PMID: 39170252 PMCID: PMC11336691 DOI: 10.1016/j.heliyon.2024.e35547] [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: 02/17/2024] [Revised: 07/30/2024] [Accepted: 07/31/2024] [Indexed: 08/23/2024] Open
Abstract
A woman in her thirties who had been diagnosed with Morbihan disease did not notice a significant improvement in her condition after receiving years of treatment. Our decision to use Baricitinib helped her to achieve a better outcome. To our knowledge, this is the first study to use Baricitinib in Morbihan disease, although JAK inhibitors have already been successfully used before. It is hoped that our case report will provide new treatment options for Morbihan disease therapy.
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Affiliation(s)
- Luying Chen
- Department of Dermatology, Seventh People's Hospital of Shanghai University of Traditional Chinese Medicine, Shanghai, China
| | - Yanjuan Duan
- Department of Dermatology, Seventh People's Hospital of Shanghai University of Traditional Chinese Medicine, Shanghai, China
| | - Liang Zhao
- Department of Dermatology, Seventh People's Hospital of Shanghai University of Traditional Chinese Medicine, Shanghai, China
| | - Miao Xu
- Department of Dermatology, Seventh People's Hospital of Shanghai University of Traditional Chinese Medicine, Shanghai, China
| | - Yeqiang Liu
- Department of Dermatopathology, Shanghai Skin Disease Hospital of Tongji University School of Medicine, Shanghai, China
| | - Xiaoxiang Zhai
- Department of Dermatology, Seventh People's Hospital of Shanghai University of Traditional Chinese Medicine, Shanghai, China
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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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Clanner-Engelshofen BM, Bernhard D, Dargatz S, Flaig MJ, Gieler U, Kinberger M, Klövekorn W, Kuna AC, Läuchli S, Lehmann P, Nast A, Pleyer U, Schaller M, Schöfer H, Steinhoff M, Schwennesen T, Werner RN, Zierhut M, Reinholz M. S2k guideline: Rosacea. J Dtsch Dermatol Ges 2022; 20:1147-1165. [PMID: 35929658 DOI: 10.1111/ddg.14849] [Citation(s) in RCA: 27] [Impact Index Per Article: 9.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/09/2022] [Accepted: 06/10/2022] [Indexed: 12/01/2022]
Abstract
This updated and upgraded S2k guideline deals with the diagnosis and treatment of rosacea, which is a common, chronic inflammatory skin disease mostly affecting the face. Initially, rosacea is characterized by recurrent erythema, telangiectasia and flushing. Later, the inflammatory component predominates, with persistent erythema with follicular papules, papulopustules and pustules. The development of phyma, which usually occurs on the acral localizations, is the most severe manifestation. For the treatment of rosacea, the interdisciplinary guideline committee, with representatives of the German Dermatological Society (DDG), the Professional Association of German Dermatologists (BVDD), the German Opthalmological Society (DOG), the Society for Dermopharmacy (GD), the Swiss Society for Dermatology and Venereology (SGDV) and the German Rosacea Aid e. V., recommends the avoidance of trigger factors and topical applications of metronidazole, azelaic acid or ivermectin. For symptomatic treatment of persistent centrofacial erythema, the topical vasoconstrictors brimonidine or oxymetazoline can also be used. Systemic therapy is recommended for therapy-resistant and severe forms of rosacea papulopustulosa. The drug of choice is low-dose doxycycline. Alternatively, low-dose isotretinoin can be recommended. Ocular rosacea should be treated with lid margin hygiene. For topical treatment, ciclosporin eye drops, azithromycin, ivermectin or metronidazole are suggested.
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Affiliation(s)
| | - Dominik Bernhard
- Department and Clinic for Dermatology and Allergology, University Hospital Munich, LMU Munich, Germany
| | - Sonja Dargatz
- Deutsche Rosazea Hilfe e.V. (German Rosacea Aid), Hamburg, Germany
| | - Michael J Flaig
- Department and Clinic for Dermatology and Allergology, University Hospital Munich, LMU Munich, Germany
| | - Uwe Gieler
- Vitos Hospital for Psychosomatic Medicine, University Hospital Gießen - Dermatology, UKGM Gießen, Gießen, Germany
| | - Maria Kinberger
- Department of Dermatology, Venereology and Allergology, Division of Evidence- Based Medicine (dEBM), Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany
| | | | - Anne-Charlotte Kuna
- Department and Clinic for Dermatology and Allergology, University Hospital Munich, LMU Munich, Germany
| | - Severin Läuchli
- Department of Dermatology, University Hospital Zurich, Zurich, Switzerland
| | - Percy Lehmann
- Center for Dermatology, Allergology and Dermatosurgery, HELIOS University Hospital Wuppertal, Witten/Herdecke University, Wuppertal, Germany
| | - Alexander Nast
- Department of Dermatology, Venereology and Allergology, Division of Evidence- Based Medicine (dEBM), Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany
| | - Uwe Pleyer
- Department of Ophthalmology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany
| | - Martin Schaller
- Department of Dermatology, University Hospital Tübingen, Eberhard Karls Universität Tübingen, Tübingen, Germany
| | - Helmut Schöfer
- German Clinic for Diagnostics (DKD, Deutsche Klinik für Diagnostik) Dermatology, Helios Kliniken Wiesbaden, Wiesbaden, Germany
| | - Martin Steinhoff
- Weill Cornell Medicine-Qatar, Cornell University, Ar-Rayyan, Qatar
| | | | - Ricardo Niklas Werner
- Department of Dermatology, Venereology and Allergology, Division of Evidence- Based Medicine (dEBM), Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany
| | - Manfred Zierhut
- Department of Ophthalmology, University Hospital Tübingen, Eberhard Karls Universität Tübingen, Tübingen, Germany
| | - Markus Reinholz
- Department and Clinic for Dermatology and Allergology, University Hospital Munich, LMU Munich, Germany
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Clanner-Engelshofen BM, Bernhard D, Dargatz S, Flaig MJ, Gieler U, Kinberger M, Klövekorn W, Kuna AC, Läuchli S, Lehmann P, Nast A, Pleyer U, Schaller M, Schöfer H, Steinhoff M, Schwennesen T, Werner RN, Zierhut M, Reinholz M. S2k-Leitlinie: Rosazea. J Dtsch Dermatol Ges 2022; 20:1147-1167. [PMID: 35971589 DOI: 10.1111/ddg.14849_g] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/09/2022] [Accepted: 06/10/2022] [Indexed: 11/28/2022]
Affiliation(s)
- Benjamin M Clanner-Engelshofen
- Klinik und Poliklinik für Dermatologie und Allergologie, Klinikum der Universität München, LMU München, München, Deutschland
| | - Dominik Bernhard
- Klinik und Poliklinik für Dermatologie und Allergologie, Klinikum der Universität München, LMU München, München, Deutschland
| | | | - Michael J Flaig
- Klinik und Poliklinik für Dermatologie und Allergologie, Klinikum der Universität München, LMU München, München, Deutschland
| | - Uwe Gieler
- Vitos-Klinik für Psychosomatik, Gießen - Universitäts-Hautklinik, UKGM Gießen, Gießen, Deutschland
| | - Maria Kinberger
- Department of Dermatology, Venereology and Allergology, Division of Evidence-Based Medicine (dEBM), Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Deutschland
| | | | - Anne-Charlotte Kuna
- Klinik und Poliklinik für Dermatologie und Allergologie, Klinikum der Universität München, LMU München, München, Deutschland
| | - Severin Läuchli
- Dermatologische Klinik, Universitätsspital Zürich, Zürich, Schweiz
| | - Percy Lehmann
- Zentrum für Dermatologie, Allergologie und Dermatochirurgie, HELIOS Universitätsklinikum Wuppertal, Universität Witten/Herdecke, Wuppertal, Deutschland
| | - Alexander Nast
- Department of Dermatology, Venereology and Allergology, Division of Evidence-Based Medicine (dEBM), Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Deutschland
| | - Uwe Pleyer
- Klinik für Augenheilkunde, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Deutschland
| | - Martin Schaller
- Universitäts-Hautklinik, Universitätsklinikum Tübingen, Eberhard Karls Universität Tübingen, Tübingen, Deutschland
| | - Helmut Schöfer
- Deutsche Klinik für Diagnostik (DKD) Dermatologie, Helios Kliniken Wiesbaden, Wiesbaden, Deutschland
| | - Martin Steinhoff
- Weill Cornell Medicine-Qatar, Cornell University, Ar-Rayyan, Qatar
| | | | - Ricardo Niklas Werner
- Department of Dermatology, Venereology and Allergology, Division of Evidence-Based Medicine (dEBM), Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Deutschland
| | - Manfred Zierhut
- Universitäts-Augenklinik, Universitätsklinikum Tübingen, Eberhard Karls Universität Tübingen, Tübingen, Deutschland
| | - Markus Reinholz
- Klinik und Poliklinik für Dermatologie und Allergologie, Klinikum der Universität München, LMU München, München, Deutschland
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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; 9:7163-7168. [PMID: 34540973 PMCID: PMC8409198 DOI: 10.12998/wjcc.v9.i24.7163] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/05/2021] [Revised: 05/31/2021] [Accepted: 07/09/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Morbihan syndrome is a rare illness, which presents with recurrent pitting edema on the upper two thirds of the face causing facial contour deformities, and Morbihan syndrome obstructing the pupillary axis with ptosis was seldomly reported.
CASE SUMMARY A 59-year-old woman presented with a 15-year history of facial swelling that progressively invaded bilaterally from the inner canthus and eyelids outwards. Imaging examination indicated that the bilateral periorbital and the left temporal soft tissues had swelling without cranium invasion. Histopathological analysis showed a large amount of lymphocyte infiltration, and immunohistochemistry showed positive expression of CD68 in clear-cut granulomas and D2-40 in the lymphatic endothelium. Finally, the clinical diagnosis of Morbihan syndrome was confirmed.
CONCLUSION Morbihan syndrome is an infrequent and refractory disease, which is characteristic with recurrent woody facial edema on the upper two thirds of the face. Solid facial edema is persistent and non-pitting, causing facial contour deformities and even vision field impairment. The diagnosis of Morbihan syndrome depends on clinical features, imaging information, and pathology. Blepharoplasty is optional to improve the visual field for these patients with severe pupillary axis obstruction.
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Affiliation(s)
- Lei Zhang
- Department of Plastic and Reconstructive Surgery, Plastic and Reconstructive Surgery Center, Zhejiang Provincial People’s Hospital, Affiliated Hospital of Hangzhou Medical College, Hangzhou 310014, Zhejiang Province, China
| | - Sheng Yan
- Department of Plastic and Reconstructive Surgery, Plastic and Reconstructive Surgery Center, Zhejiang Provincial People’s Hospital, Affiliated Hospital of Hangzhou Medical College, Hangzhou 310014, Zhejiang Province, China
| | - Lei Pan
- Department of Plastic and Reconstructive Surgery, Plastic and Reconstructive Surgery Center, Zhejiang Provincial People's Hospital, People's Hospital of Hangzhou Medical College, Hangzhou 310014, Zhejiang Province, China
| | - Su-Fan Wu
- Department of Plastic and Reconstructive Surgery, Plastic and Reconstructive Surgery Center, Zhejiang Provincial People’s Hospital, Affiliated Hospital of Hangzhou Medical College, Hangzhou 310014, Zhejiang Province, China
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Abstract
ABSTRACT Morbihan disease is a rare condition characterized by persistent erythema and solid edema of the upper two thirds of the face, leading to visual impairment and significant esthetic disfigurement. The underlying etiology of the disease remains uncertain, but its correlation with lymphoedema has been conjectured. Definitive treatment options are also lacking, and most current topical, systemic, and surgical interventions provide transient or partial results.In this report, we describe a case of Morbihan disease in a 32-year-old man. He suffered from erythema and swelling over the left periorbital region for 6 years, and was diagnosed with Morbihan disease. As the edema was unresponsive to drug therapy, he was referred to our department. Indocyanine green lymphography was performed, and the diagnosis of facial lymphoedema was confirmed. Manual lymphatic drainage was not effective, so we proceeded with surgical procedures. Lymphaticovenous anastomosis was performed to improve lymphatic drainage, and blepharoplasty was performed to debulk the enlarged tissue of lymphoedema. The visual field improved significantly, and the patient remains free from reexpansion of the lesion during the 1-year follow-up.The surgical approach for lymphoedema treatment can be a viable option for severe Morbihan disease unresponsive to nonsurgical therapies.
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Affiliation(s)
| | - Haruko Hino
- Department of Dermatology, Kanto Central Hospital of the Mutual Aid Association of Public School Teachers, Kamiyoga, Setagaya-ku, Tokyo Japan
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Chon BH, Hwang CJ, Perry JD. Treatment Options for Lower Eyelid Festoons. Facial Plast Surg Clin North Am 2021; 29:301-309. [PMID: 33906762 DOI: 10.1016/j.fsc.2021.02.005] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/21/2022]
Abstract
Festoons represent a combination of fluid accumulation and soft tissue laxity in the superolateral cheek. They remain a difficult entity to treat. The ideal treatment for festoons would possess minimal invasiveness and recovery time, and predictably improve the condition. No nonsurgical treatment currently meets these criteria, and surgical treatments have significant limitations. Fortunately, a variety of treatment options exist that can benefit each patient and be tailored to their specific needs. Knowledge of the underlying anatomy, clinical characteristics, and clinical evaluation will better equip the treating physician to manage festoons.
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Affiliation(s)
- Brian H Chon
- Oculofacial Plastic Surgery, Department of Ophthalmology, Cole Eye Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195, USA
| | - Catherine J Hwang
- Oculofacial Plastic Surgery, Department of Ophthalmology, Cole Eye Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195, USA
| | - Julian D Perry
- Oculofacial Plastic Surgery, Department of Ophthalmology, Cole Eye Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195, USA.
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Abstract
Facial edemas not secondary to surgery and/or radiotherapy for head and neck cancer are relatively uncommon. Our aim is to report a retrospective analysis of the lymphoscintigraphic and SPECT-CT investigations obtained in patients with such facial edema. Retrospective review of exams (planar imagings in all and with SPECT-CT in 5) obtained after the subcutaneous injection of 99mTc HSA Nanosized colloids between the eyebrows in five men and seven women. Four main lymphatic pathways were identified on sequential planar imagings: para-nasal left and right and supra- ocular left and right. For eleven patients, the absence of visualization of lymphatic drainage and/or their delayed appearance correlated well with the localisation of the edematous areas. In two patients with post-traumatic and post- surgical edemas, SPECT-CT showed one deep left sided cervical lymph node (LN) in front of the first cervical vertebra. This lymphoscintigraphic approach represents a simple and valuable way to assess the lymphatic drainage pathways of the face and to establish the diagnosis of facial lymphedema.
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A Navajo Patient with Morbihan's Disease: Insight into Oculoplastic Treatment of a Rare Disease. PLASTIC AND RECONSTRUCTIVE SURGERY-GLOBAL OPEN 2020; 8:e3090. [PMID: 33133943 PMCID: PMC7544268 DOI: 10.1097/gox.0000000000003090] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/21/2020] [Accepted: 07/15/2020] [Indexed: 11/25/2022]
Abstract
Morbihan’s disease is classically defined as primarily a Caucasian disease of chronic, recurrent erythema and nonpitting edema of the middle and upper thirds of the face. The disease is often difficult to diagnose and challenging to treat. Medical management is the primary treatment modality; however, if there is progression of the disease that causes visual impairment, surgical debulking is often required. We present the first reported case of Morbihan’s disease in a Navajo patient in conjunction with a review of the literature on surgical management of this challenging disease.
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Clinicopathological and Immunohistochemical Study of 14 Cases of Morbihan Disease: An Insight Into Its Pathogenesis. Am J Dermatopathol 2020; 41:701-710. [PMID: 31567295 DOI: 10.1097/dad.0000000000001378] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/06/2023]
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.
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Unilateral Periocular Intralymphatic Histiocytosis, Associated With Rosacea (Morbihan Disease). Am J Dermatopathol 2019; 42:452-454. [DOI: 10.1097/dad.0000000000001567] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
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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. [PMID: 30561675 DOI: 10.1093/ptj/pzy155] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/19/2018] [Accepted: 09/16/2018] [Indexed: 02/09/2023]
Abstract
BACKGROUND AND PURPOSE Morbihan disease, also known as rosacea lymphedema, is a rare persistent form of lymphedema that is associated with the disease rosacea. Even though acne rosacea responds well to standard medical treatment, the lymphedema component of the disease is resistant to both medical and surgical therapy. Complete decongestive therapy (CDT) can be considered as a conservative alternative option for treatment of rosacea lymphedema. To date, there is no report on the use of CDT in treating facial lymphedema secondary to acne rosacea. CASE DESCRIPTION We present 2 cases of women with a diagnosis of Morbihan disease and chronic facial lymphedema that remained resistant to drug treatment for many years before CDT was offered. The treatment program included 4 components: manual lymphatic drainage, compression bandaging, exercises to enhance lymphatic drainage, and patient education. OUTCOMES Following 10 to 15 sessions of CDT, the first patient's facial edema had almost completely resolved. The second patient's response to treatment was assessed as moderate. DISCUSSION To the best of our knowledge, these 2 cases of Morbihan disease treated with CDT are the first of their kind to be presented in the literature. As the treatment options for Morbihan disease remain inadequate, we believe that CDT should be considered as a treatment option in those patients who do not benefit from or refuse drug treatment, before moving on to more invasive procedures. Prospective studies should be designed to demonstrate the efficacy of CDT and provide management details.
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Affiliation(s)
- Sehim Kutlay
- Department of Physical and Rehabilitation Medicine, Ankara University, Ankara, Turkey
| | - Elif Can Ozdemir
- Department of Physical and Rehabilitation Medicine, Ankara University, and İbn-i Sina Hospital Altındağ, Ankara 06100, Turkey
| | - Zahide Pala
- Department of Physical and Rehabilitation Medicine, Ankara University
| | - Selin Ozen
- Department of Physical and Rehabilitation Medicine, Baskent University, Ankara, Turkey
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15
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Morbihan Disease Treatment: Two Case Reports and a Systematic Literature Review. Ophthalmic Plast Reconstr Surg 2019; 35:126-132. [DOI: 10.1097/iop.0000000000001229] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/13/2023]
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16
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Abstract
BACKGROUND Cutaneous angiosarcoma presents clinically in numerous ways, and can be mistaken for a different clinical entity, particularly when arising at unusual anatomic locations such as the eyelid. CASE PRESENTATION A 57-year-old woman presented with a 1-year history of eyelid swelling. Concurrent imaging was also suggestive of an edematous process. Multiple superficial biopsies showed nonspecific dermal inflammation and interstitial edema. A diagnosis of Morbihan disease (chronic and idiopathic lymphedema of the eyelid) was rendered, and the patient was treated with compression and local therapy without clinical improvement. Three years after initial presentation, a diagnostic blepharoplasty was performed revealing a deep dermal vascular proliferation composed of anastomosing vascular channels with an atypical endothelial lining. A diagnosis of cutaneous angiosarcoma was ultimately made. CONCLUSIONS This case illustrates a unique presentation of cutaneous angiosarcoma and the implications of different biopsy techniques in acquiring the correct diagnosis.
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17
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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. [PMID: 29687653 PMCID: PMC6175370 DOI: 10.1111/dth.12609] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/17/2017] [Revised: 11/12/2017] [Accepted: 03/18/2018] [Indexed: 01/29/2023]
Abstract
Morbihan's disease is characterized by chronic persistent facial edema of the upper half of the face, absence of typical diagnostic findings, and refractoriness to treatment. A 44-year-old man was diagnosed with Morbihan's disease based on clinical signs and histopathology, which showed dermal edema in upper dermis, discrete lymphocytic infiltrate without granulomatous reaction, and mast cell infiltration. After long-term therapy with intralesional triamcinolone a remarkable objective and subjective clinical response was observed. Reported cases of Morbihan's disease are reviewed, with respect to their treatment and histopathological findings. Mast cell infiltration has been observed on histopathology in most patients who responded to intralesional triamcinolone, suggesting a possible marker of response. The long-lasting response seen in our case indicates the efficacy of intralesional triamcinolone in this rare condition.
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Affiliation(s)
- Aikaterini Tsiogka
- Dermatology department, University Hospital of Paracelsus, Medical University of Salzburg, Salzburg, Austria
| | - Josef Koller
- Dermatology department, University Hospital of Paracelsus, Medical University of Salzburg, Salzburg, Austria
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18
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Anzengruber F, Czernielewski J, Conrad C, Feldmeyer L, Yawalkar N, Häusermann P, Cozzio A, Mainetti C, Goldblum D, Läuchli S, Imhof L, Brand C, Laffitte E, Navarini AA. Swiss S1 guideline for the treatment of rosacea. J Eur Acad Dermatol Venereol 2017; 31:1775-1791. [PMID: 28833645 DOI: 10.1111/jdv.14349] [Citation(s) in RCA: 32] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/20/2016] [Accepted: 05/08/2017] [Indexed: 12/28/2022]
Abstract
Rosacea (in German sometimes called 'Kupferfinne', in French 'Couperose' and in Italian 'Copparosa') is a chronic and frequently relapsing inflammatory skin disease primarily affecting the central areas of the face. Its geographic prevalence varies from 1% to 22%. The differential diagnosis is wide, and the treatment is sometimes difficult and varies by stage of rosacea. For erythematous lesions and telangiectasia, intense pulsed light (IPL) therapy and lasers are popular treatment option. In addition, a vasoconstrictor agent, brimonidine, has recently been developed. For papulopustular rosacea, topical antibiotics, topical and systemic retinoids, as well as systemic antibiotics are used. A topical acaricidal agent, ivermectin, has undergone clinical development and is now on the market. In the later stages, hyperplasia of the sebaceous glands develops, resulting in phymatous growths such as the frequently observed bulbous nose or rhinophyma. Ablative laser treatments have largely replaced classical abrasive tools. Here, we reviewed the current evidence on the treatment of rosacea, provide a guideline (S1 level) and discuss the differential diagnosis of rosacea.
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Affiliation(s)
- F Anzengruber
- Department of Dermatology, University Hospital Zurich, Zurich, Switzerland
| | - J Czernielewski
- Department of Dermatology, University Hospital Lausanne, Lausanne, Switzerland
| | - C Conrad
- Department of Dermatology, University Hospital Lausanne, Lausanne, Switzerland
| | - L Feldmeyer
- Department of Dermatology, Inselspital, University Hospital Bern, University of Bern, Bern, Switzerland
| | - N Yawalkar
- Department of Dermatology, Inselspital, University Hospital Bern, University of Bern, Bern, Switzerland
| | - P Häusermann
- Department of Dermatology, University Hospital Basel, Basel, Switzerland
| | - A Cozzio
- Department of Dermatology, Kantonsspital St. Gallen, St. Gallen, Switzerland
| | - C Mainetti
- Department of Dermatology, Ospedale Regionale di Bellinzona e Valli, Bellinzona, Switzerland
| | - D Goldblum
- Department of Ophthalmology, University Hospital Basel, Basel, Switzerland
| | - S Läuchli
- Department of Dermatology, University Hospital Zurich, Zurich, Switzerland
| | - L Imhof
- Department of Dermatology, University Hospital Zurich, Zurich, Switzerland
| | - C Brand
- Department of Dermatology, Lucerne Cantonal Hospital, Lucerne, Switzerland
| | - E Laffitte
- Department of Dermatology, University Hospital Geneva, Geneva, Switzerland
| | - A A Navarini
- Department of Dermatology, University Hospital Zurich, Zurich, Switzerland
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