A case report on Madelung's disease and comprehensive review of the literature.

BACKGROUND: Madelung's disease (MD), alternatively referred to as benign symmetric lipomatosis, multiple symmetric lipomatosis, or Launois-Bensaude syndrome, is an uncommon benign disorder marked by symmetric proliferation of adipose tissue in the head, neck, and torso regions. CASE DESCRIPTION: In this case, the patient was a female with the late middle-aged demographic, diagnosed with Type I MD. Notably, she exhibited no prior history of alcohol consumption, and there was no family history of MD. Subsequent to the clinical diagnosis, the patient underwent medical imaging assessments to delineate the surgical parameters. Post-surgery, she demonstrated a favorable recovery trajectory, marked by the absence of any surgical complications. RESULT: The patient underwent successful surgical excision of the lipomatous mass. Postoperatively, she had an uneventful recovery with no complications and no recurrence observed during the follow-up period of seven months. CONCLUSION: Timely diagnosis and early surgical intervention play a pivotal role in enhancing the quality of life for individuals with MD. Preoperative medical imaging examinations function as highly effective tools, contributing to heightened surgical safety and a decreased probability of encountering complications during the surgical procedure.

A Chinese patient with Madelung's disease and alcoholic cardiomyopathy: a case report and literature review.

BACKGROUND: Madelung's disease is a rare disorder characterized by massive deposits of excess subcutaneous fat around the neck, shoulders, arms, and other parts of the body. It has a high prevalence among middle-aged alcoholic men in Mediterranean countries and a low incidence in Asian populations. Although patients with Madelung's disease are often associated with a variety of alcohol-induced metabolic disorders, the comorbidity of alcoholic cardiomyopathy is rarely reported, probably because of its low incidence or neglect by clinicians. CASE REPORT: A 67-year-old man with a 10-year history of soft fat masses in the neck developed chest tightness and shortness of breath on exertion for the past 2 years. Laboratory tests revealed elevated γ-glutamyl transferase, glucose intolerance, hyperuricemia, hyperlipidemia, and anemia. Computed tomography of the neck showed symmetric nonencapsulated fat deposits, mainly in the anterior cervical regions. Echocardiography showed left heart enlargement and severe global left ventricular systolic dysfunction with an ejection fraction of 31%. Coronary angiography revealed 40-50% stenoses of the left anterior descending and right coronary arteries. After the exclusion of other causes of dilated cardiomyopathy, the patient was finally diagnosed with type I Madelung's disease and alcoholic cardiomyopathy. He underwent lifestyle changes, including reducing his alcohol intake, and received full pharmacological treatment for heart failure. One and a half years later, his cardiac function was partially restored, and all metabolic abnormalities improved except for elevated liver enzymes. CONCLUSIONS: Alcohol use disorder should be assessed in patients with newly diagnosed Madelung's disease. Screening for alcoholic cardiomyopathy in alcoholic patients with Madelung's disease is necessary for early detection of cardiac abnormalities and intervention to improve the prognosis of this group of patients.

Lipomatoses.

Lipomatoses are benign proliferation of adipose tissue. Lipomas (benign fat tumors) are the most common component of lipomatosis. They may be unique or multiple, encapsulated or not, subcutaneous or sometimes visceral. In some cases, they form large areas of non-encapsulated fat hypertrophy, with a variable degree of fibrosis. They can develop despite the absence of obesity. They may be familial or acquired. At difference with lipodystrophy syndromes, they are not associated with lipoatrophy areas, except in some rare cases such as type 2 familial partial lipodystrophy syndromes (FPLD2). Their metabolic impact is variable in part depending on associated obesity. They may have functional or aesthetic consequences. Lipomatosis may be isolated, be part of a syndrome, or may be visceral. Isolated lipomatoses include multiple symmetrical lipomatosis (Madelung disease or Launois-Bensaude syndrome), familial multiple lipomatosis, the painful Dercum's disease also called Adiposis Dolorosa or Ander syndrome, mesosomatic lipomatosis also called Roch-Leri lipomatosis, familial angiolipomatosis, lipedema and hibernomas. Syndromic lipomatoses include PIK3CA-related disorders, Cowden/PTEN hamartomas-tumor syndrome, some lipodystrophy syndromes, and mitochondrial diseases, especially MERRF, multiple endocrine neoplasia type 1, neurofibromatosis type 1, Wilson disease, Pai or Haberland syndromes. Finally, visceral lipomatoses have been reported in numerous organs and sites: pancreatic, adrenal, abdominal, epidural, mediastinal, epicardial… The aim of this review is to present the main types of lipomatosis and their physiopathological component, when it is known.

Perineal Benign Symmetric Lipomatosis in a Female Patient: A Case Report.

BACKGROUND Benign symmetric lipomatosis (BSL), also known as Madelung's disease, is a rare disease generally characterized by fat deposits painlessly and symmetrically distributed in the body. In former studies, the incidence of BSL is highest in male patients and more frequent in the Mediterranean area. Asian females seem to be rarely affected. BSL often occurs in the neck or upper-back of patients, and is related to various metabolic disorders. Unusual clinical appearances may add difficulties in diagnosis. CASE REPORT In this report, we present a case of BSL in a 33-year-old woman's perineal region, with no clear BSL risk factors (sex, medical history, sites, and comorbidities), which increased the difficulties in diagnosis. The patient's quality of life was seriously affected by the continuous growth of fat tissue. Based on MRI and B-ultrasonic examinations, she underwent excision at our outpatient facility. Combined with the patient's clinical appearance, imaging results, and pathological tests, we could finally determine the diagnosis of BSL. After 18 months of follow-up, this patient recovered well with no recurrence. CONCLUSIONS Difficulties in diagnosis can seriously affect doctors' treatment approaches. BSL rarely occurs in the lower body, and our patient showed no clear risk factors. Therefore, imaging and pathological examinations can be essential tools for dermatological and plastic surgeons to diagnose and treat rare BSL.

Multiple symmetrical lipomatosis: A literature review and case report of a patient with Madelung's disease.

<b><br>Introduction:</b> Madelung's disease is a rare condition characterised by the symmetric growth of fatty tumours (lipomas) around the neck, shoulders, upper arms and trunk.</br> <b><br>Case report:</b> We present a description of a male patient with extensive adipose tissue overgrowth around the neck. Once the possibility of malignancy was excluded, the patient's history and clinical and radiological findings led to the diagnosis of Madelung's disease. A two-stage surgery was planned and the patient underwent lipectomy of the lipomas around the neck.</br> <b><br>Conclusions:</b> This article analyses the clinical data with Madelung's disease; discusses its aetiology, clinical manifestations, diagnosis and treatment methods; and provides help with clinical diagnosis and treatment.</br>.

Madelung's Disease: A Rare Cause of Dyspnea

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Madelung disease (multiple symmetric lipomatosis)

Madelung disease or multiple symmetric lipomatosis (MSL) is a rare entity among the overgrowth syndromes. It is characterized by painless non-encapsulated and symmetric fatty deposits in the neck, torso, mammary, and abdominal areas, and in the upper and lower limbs. The etiology of the disease is still unknown. Chronic alcohol consumption may play a role in adipocyte hyperplasia in genetically susceptible individuals. Besides the overgrowth of adipose tissue, patients with MSL present features of metabolic syndrome. Patients seek medical attention usually for esthetic reasons. We present the case of a middle-aged man who sought the outpatient clinic complaining of bulging masses in the posterior upper part of the thorax, the occipital area, and the neck. The masses grew over a period of 2 years. The physical examination and imaging study revealed the presence of symmetric lipomatosis. A two-step surgical treatment was undertaken for the excision of the lipomatous tissue. The postoperative outcome was uneventful with satisfactory esthetic results.

Macroglosia secundaria a enfermedad de Madelung / Macroglossia in Madelung's disease

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Deformidad de Madelung / Madelung’s deformity

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Lipomatosis benigna simétrica; síndrome de Madelung / Symmetric benign lipomatosis; Madelung syndrome

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Enfermedad de Madelung / Madelung’s disease

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Lipoma cervical gigante: a propósito de un caso / Giant cervical lipoma: a case report

Los lipomas son los tumores mesenquimales más frecuentes. La aparición de lipomas gigantes a nivel cervical es infrecuente. Es necesaria la realización de pruebas de imagen para una correcta localización anatómica y establecer relaciones con las estructuras vecinas. El tratamiento de elección es la escisión quirúrgica de la masa. Presentamos el caso de un varón de 59 años que presenta una tumoración gigante cervical de 10 años de evolución. La planificación prequirúrgica en este tipo de casos debe ser exquisita para garantizar la conservación tanto de la función como de la estética cervicofacial(AU)

Lipomas are the most common mesenchymal tumors, although giant lipomas of the neck region are uncommon. Imaging is needed to correctly identify the anatomic location of the tumor and its relation to surrounding structures. Surgical excision is the treatment of choice. The case of a 59-year-old man with a giant neck tumor of 10 years evolution is reviewed. Meticulous preoperative planning is required in these cases to ensure a cosmetically and functionally acceptable cervicofacial outcome(AU)

Lipoma laríngeo asociado a enfermedad de Madelung: a propósito de un caso / Laryngeal lipoma associated with Madelung’s disease: a case report

La lipomatosis cervical múltiple es una alteración del depósito graso cervical, parte alta de tórax y extremidades superiores que genera un problema estético y eventualmente cuadros obstructivos de la vía respiratoria en aquellos casos de infiltración grasa de la laringe. Presentamos el caso de un hombre con enfermedad de Madelung y cuadro disneico agudo por infiltración grasa laríngea y lipoma obstructivo (AU)

Multiple symmetric lipomatosis is an alteration in the neck, upper trunk and upper extremities fat deposits. It produces an aesthetic problem and sometimes upper airway obstruction when the larynx is infiltrated by the mass. We report the case of a male with Madelung's disease, which began with acute dyspnea caused by laryngeal fat deposits and obstructive lipoma (AU)

Enfermedad de Madelung / Madelung's disease

La enfermedad de Madelung o lipomatosis simétrica múltiple es una enfermedad rara caracterizada por la presencia de masas de tejido adiposo, distribuidas de forma simétrica en cuello, nuca, tronco, hombros y parte proximal de los miembros. Afecta fundamentalmente a hombres entre los 30 y 60 años con historia de etilismo crónico, siendo la resección quirúrgica el único tratamiento efectivo (AU)

Madelung's disease or Benign Symmetric Lipomatosis is a rare disease characterised by masses of adipose tissue spread symmetrically along the neck, trunk, shoulders and upper arms. It affects men from 30 to 60 years old with a previous history of alcoholism. Surgery is the only effective treatment (AU)

Doença de Madelung: relato de caso e revisão da literatura / Madelung's disease: a case report and literature review

A doença de Madelung é caracterizada por massas simétricas de tecido adiposo no pescoço, ombros, braços e parte superior do tronco. A tomografia computadorizada demonstra achados de imagem característicos e é considerada o método de escolha para diagnóstico, estadiamento pré-operatório e acompanhamento pós-cirúrgico. Relatamos o caso de um homem com aspectos tomográficos típicos da doença de Madelung.

Madelung's disease is characterized by the presence of symmetric masses of adipose tissue on the neck, shoulders, arms and the upper trunk. Computed tomography demonstrates characteristic imaging findings, and it is considered to be the method of choice for diagnosis, pre-operative staging, and postoperative follow-up. The authors report the case of a man with typical tomographic findings of Madelung's disease.