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Table of Contents
CORRESPONDENCE
Year : 2021  |  Volume : 39  |  Issue : 1  |  Page : 51-52

A rare case of Madelung's disease in Asian female


1 Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Ishikawa, Japan
2 Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University; Department of Plastic Surgery, Kanazawa University Hospital, Kanazawa, Ishikawa, Japan

Date of Submission18-May-2020
Date of Decision09-Jul-2020
Date of Acceptance24-Jul-2020
Date of Web Publication12-Feb-2021

Correspondence Address:
Dr. Takashi Matsushita
Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, 13-1 Takara-Machi, Kanazawa, Ishikawa 920-8641
Japan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ds.ds_47_20

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How to cite this article:
Mizumaki K, Kawase M, Numata N, Okamura A, Komuro A, Matsushita T. A rare case of Madelung's disease in Asian female. Dermatol Sin 2021;39:51-2

How to cite this URL:
Mizumaki K, Kawase M, Numata N, Okamura A, Komuro A, Matsushita T. A rare case of Madelung's disease in Asian female. Dermatol Sin [serial online] 2021 [cited 2021 Apr 15];39:51-2. Available from: https://www.dermsinica.org/text.asp?2021/39/1/51/309203



Dear Editor,

Madelung's disease is a rare disease primarily characterized by multiple, symmetric, large subcutaneous fatty masses located around the neck, shoulder, back, or chest. The incidence of Madelung's disease is remarkably low in the Asian populations.[1] Previously, a typical case of Madelung's disease in a Taiwanese man was reported in 1996.[2] Herein, we report a rare case of a Japanese woman with Madelung's disease.

A 50-year-old Japanese woman presented to the dermatology department of our hospital with a 5-year history of bulging in the bilateral upper arms that had gradually expanded. There were no signs of pain or sensory disorder, and her movements were largely unrestricted. She reported >30 years of alcohol abuse and had a history of alcoholic cirrhosis and hyperlipidemia. There was no family history of her condition. Physical examination showed presence of painless bilateral masses of soft consistency in the upper back region, shoulders, and upper arms [Figure 1]a and [Figure 1]b. Computed tomography confirmed the presence of extensive masses with fat density in the referred area. Magnetic resonance imaging was not performed due to severe claustrophobia. Laboratory analysis revealed elevated liver enzyme levels and hyperlipidemia. The levels of hormones and all laboratory markers of diabetes were within the normal range. Histopathological examination of the upper arm showed normal fatty tissue without any signs of malignancy [Figure 1]c and [Figure 1]d. Therefore, she was diagnosed with Madelung's disease. Post-diagnosis, she was advised to quit alcohol. Since she wanted to improve the cosmetic appearance, the bilateral masses in her shoulders and upper arms were surgically removed. Intraoperatively, the fatty masses were nonencapsulated and appeared different from regular subcutaneous fat. The fatty masses had invaded the regular subcutaneous fat around them. The deep and superficial fatty masses were resected from wherever possible. There was no recurrence up to 3 years postoperatively.{Figure 1}

Madelung's disease is also termed as multiple symmetric lipomatosis, benign symmetrical lipomatosis, or  Launois-Bensaude syndrome More Details.[3] Majority of the masses are located in the neck, shoulders, and upper arms. It has been reported that mediastinum structures are deeply infiltrated, with compression of major vessels, nerves, trachea, bronchi, and esophagus, which leads to dyspnea, dysphagia, and dysphonia.[4] Madelung's disease predominantly occurs in males, especially those aged 30–60 years; the male-to-female ratio is 15–30:1,[5] and this disease is rarely reported in Asia. Thus, an Asian female case, such as ours, is extremely rare.

Madelung's disease diagnosis depends on clinical symptoms, patient history, biopsy findings, and laboratory and imaging studies. Key differential diagnoses of Madelung's disease include morbid obesity, Cushing syndrome, myxoid liposarcoma, lymphoma, neurofibromatosis, encapsulated lipoma, angiolipoma, hibernoma, Dercum's disease, Froelich syndrome, and drug-induced lipomatosis.

Although Madelung's disease etiology still remains unclear, inefficient fat metabolism is considered to be an underlying cause. In addition, Madelung's disease patients frequently have a history of alcohol abuse and metabolic abnormalities, such as hypertension and insulin resistance.

Specific treatment for Madelung's disease has not yet been established. Surgical resection or suction-assisted lipectomy is the only effective treatment available. However, incomplete resection often leads to disease recurrence.[5] Madelung's disease is a benign disease and its malignant transformation is extremely rare. However, liposarcoma-like progression of Madelung's disease has been reported in a few cases.[6],[7] Therefore, regular follow-up is important for early diagnosis.

In conclusion, we reported the case of a Japanese woman with Madelung's disease with >30 years of alcohol abuse and a history of alcoholic cirrhosis and hyperlipidemia. Although the number of cases with Madelung's disease has been gradually increasing, in some cases, its diagnosis is often delayed. The occurrence of Madelung's disease is remarkably rare in Asian women. However, the clinicians should still consider the possibility of Madelung's disease in Asian women who present with relevant characteristic symptoms and a history of alcohol abuse and metabolic abnormalities.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.



Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Chuang CC, Cheng YF, Chang HP, Lin CZ. Madelung's disease. J Chin Med Assoc 2004;67:591-4.  Back to cited text no. 1
    
2.
Chen JY, Chao SC, Lee JY. Madelung's disease. Dermatol Sinica 1996;14:89-94.  Back to cited text no. 2
    
3.
Nisi G, Sisti A. Images in clinical medicine. Madelung's disease. N Engl J Med 2016;374:572.  Back to cited text no. 3
    
4.
Meningaud JP, Pitak-Arnnop P, Bertrand JC. Multiple symmetric lipomatosis: Case report and review of the literature. J Oral Maxillofac Surg 2007;65:1365-9.  Back to cited text no. 4
    
5.
González-García R, Rodríguez-Campo FJ, Sastre-Pérez J, Muñoz-Guerra MF. Benign symmetric lipomatosis (Madelung's disease): Case reports and current management. Aesthetic Plast Surg 2004;28:108-12.  Back to cited text no. 5
    
6.
Tizian C, Berger A, Vykoupil KF. Malignant degeneration in Madelung's disease (benign lipomatosis of the neck): Case report. Br J Plast Surg 1983;36:187-9.  Back to cited text no. 6
    
7.
Borriello M, Lucidi A, Carbone A, Iannone V, Ferrandina G. Malignant transformation of Madelung's disease in a patient with a coincidental diagnosis of breast cancer: A case report. Diagn Pathol 2012;7:116.  Back to cited text no. 7
    




 

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