Relato de caso: doença de Madelung / Case report: Madelung's disease

A síndrome de Madelung ou também conhecida por lipomatose simétrica múltipla (LSM) é uma lipodistrofia benigna rara que se caracteriza por múltiplas massas adiposas não encapsuladas. Ainda de etiologia desconhecida, existe a suspeita de uma possível herança autossômica dominante. Está fortemente associada ao abuso de álcool (60-90% dos casos). Apresenta curso clínico variável, de crescimento rápido com posterior estabilização ou progressão lenta. Pode ser assintomática ou causar sintomas de compressão de estruturas mediastinais e do trato aerodigestivo. O tratamento de escolha é cirúrgico (AU)

Madelung syndrome, also known as multiple symmetric lipomatosis (MSL), is a rare benign lipodystrophy that is characterized by multiple non-encapsulated adipose masses. Although of unknown etiology, there is a suspected autosomal dominant inheritance. It is strongly associated with alcohol abuse (60-90% of cases). It presents a variable clinical course, of fast growth with later stabilization or slow progression. It may be asymptomatic or cause symptoms of compression of mediastinal structures and aerodigestive tract. The treatment of choice is surgical (AU)

Multiple Symmetric Lipomatosis (Madelung's Disease) Presenting as Bilateral Huge Gynecomastia / 한국유방암학회지

Multiple symmetric lipomatosis (MSL), or Madelung's disease, is a rare disease of unknown etiology. It is characterized by the presence of loose adipose tissue deposits localized in the cervical region and upper body. MSL presenting as bilateral huge gynecomastia is an extremely rare phenomenon. The present report describes a case of MSL in a 66-year-old man. The patients presented with bilateral breast bulging. He had a history of cigarette and alcohol use. His condition was treated with a bilateral nipple-sparing mastectomy. MSL can present as a form of gynecomastia, for its accurate diagnosis and proper treatment of MSL, increasing awareness of the clinical characteristics of the disease is required, especially amongst breast surgeons. Herein, we review the literature and discuss the clinical characteristics, pathology, and surgical treatment of MSL.

Lipomatose simétrica múltipla / Multiple symmetric lipomatosisis

A lipomatose simétrica múltipla ou síndrome de Madelung é uma doença rara, caracterizada por depósitos de tecido adiposo de caráter benigno, de rápido crescimento, principalmente em região cervical, dorso e, mais raramente, membros e face, em geral relacionados ao alcoolismo crônico. O tratamento cirúrgico é paliativo e a recidiva é comum. Neste trabalho é relatado um caso de paciente portador de lipomatose simétrica múltipla, seguido de revisão de literatura enfocando diagnóstico e tratamento dessa afecção.

Multiple symmetric lipomatosis, also called Madelung's syndrome, is a rare disease characterized by the accumulation of rapidly growing benign fat tissue, particularly in the cervical region, back, and more rarely in the extremities and face. It is generally associated with chronic alcoholism. Surgical treatment is palliative, and relapse is common. This paper reports the case of a patient with multiple symmetric lipomatosis followed by a review of the literature addressing the diagnosis and treatment of this condition.

Doença de Madelung. Relato de caso / Madelung's disease. Case report

JUSTIFICATIVA E OBJETIVOS: A doença de Madelung, também conhecida como lipomatose simétrica benigna (LSB), é um tipo raro de lipodistrofia que se caracteriza por deposição simétrica de tecido adiposo no pescoço, porção superior do tronco e membros, determinando um desfiguramento progressivo do paciente. De etiologia desconhecida, a doença acomete principalmente homens, etilistas e, embora usualmente assintomática, pode causar complicações graves, tais como compressão traqueal, laríngea ou mediastinal. O objetivo deste estudo foi relatar um caso de doença de Madelung, bem como sua possível patogênese e tratamento. RELATO DO CASO: Paciente do sexo masculino, 45 anos, etilista, cardiopata, com lipomas distribuídos simetricamente na região cervical e superior do tórax. Exames gerais sem alterações e tomografia cervical com diminuição discreta da luz traqueal. CONCLUSÃO: A LSB, relatada como doença benigna, pode originar massas volumosas e sintomas compressivos de vias aéreas, conferindo prognóstico reservado. Ressalta-se a importância da suspeição diagnóstica, que é feita à ectoscopia.

BACKGROUND AND OBJECTIVES: Madelung's disease, also known as benign symmetric lipomatosis (BSL), is a rare type of lipodystrophy characterized by symmetric deposits of adipous tissue in neck, upper trunk and limbs, determinating a progressive disfigurement of the patient. Of unknown etiology, this disease occurs usually in men, alcoholist and, despite asymptomatic, it may lead to severe complication - as tracheal, laryngeal and mediastinal compression. The objective of this study was report a Madelung's disease case, as well as the possible pathogenesis and treatment. CASE REPORT: An alcoholist and cardiopatic 45 year-old male patient, with symmetric lipomas in the cervical region and upper part of trunk, and a light tracheal compression, shown in cervical tomography. CONCLUSION: The BSL, although considered a benign disease, it can generate bulky masses and airways compression symptoms, and so a bad prognosis. Because of it, the clinical suspection becomes important. It is suggested by ectoscopy.

Bilateral Vocal Cord Palsy Associated with Multiple Symmetrical Lipomatosis(Madelung's Disease) / 대한이비인후과학회지

Multiple symmetrical lipomatosis (MSL) is a rare disorder characterized by the presence of multiple, symmetric, unencapsulated fat masses in the face, neck, and other areas. Typically, this entity has been related to the presence of three anterior bulges in the neck. MSL is usually described in adults from 30 to 60 years old, with an incidence of about 1 in 25,000 and a male-to-female ratio of 15 : 1 to 30 : 1. More than 90% of the patients have associated alcoholism. The etiology of MSL remains unknown, but an abnormal lipogenesis induced by catecholamines has been observed. The accumulation of fat prevents accurate clinical assessment of the neck and obscures other underlying abnormalities. In the current report, the authors described one case of MSL associated with bilateral vocal cord palsy and reviewed the associated literature.

A Case of Symmetrical Lipomatosis of the Tongue Presenting as Macroglossia / Oral Science International

Symmetrical lipomatosis is characterized by symmetrical infiltration of adipose tissue into the head, neck, or upper trunk. By contrast, oral involvement is extremely rare. The present report describes a case of symmetrical lipomatosis of the tongue (SLT) presenting as macroglossia. The patient was a 63-year-old Japanese male who developed progressive dysarthria secondary to enlargement of the tongue. The patient's medical history was notable for alcoholic hepatitis and hyperlipidemia. Marginal glossectomy was performed on both sides of the tongue to achieve volume reduction and restore normal speech. Histopathologic examination of the resected tissue revealed diffuse infiltration with adipose tissue lacking a fibrous capsule. The patient's postoperative course was uneventful, and he remained free of recurrence or regrowth and functional deficits at the 6-year follow-up time point.

Two Cases of Benign Symmetrical Lipomatosis / 대한피부과학회지

We report two cases of benign symmetrical lipornatosis, one associated with mild hyperthyroidism in a 57-year-old man and the other with alcoholic hepatitis in a 57-year-old man. In the histologic exsmination of the excised mass we found fibrous pseudocapsules surrounding adipose tissue in case 1, but not in case 2. Liposuction was performed in case 1 and simple excision in case 2 for treatment of tumors. The tumor masses were not recurred in clinical observation for follow-up 5 months after treatment.