1/11. A case of multiple symmetrical lipomatosis (Madelung's disease).Multiple symmetrical lipomatosis (Madelung's disease) is a rare disease with multiple symmetrical unencapsulated fatty accumulation diffusely involving the neck, the shoulders and the upper extremities (Kohan et al. Otolaryngol. head neck Surg. 1993;108:156-159). We describe a 48-year-old Japanese man with a history of alcoholism and liver cirrhosis who reported gradually enlarging masses in his cervical region for 4 years. MRI revealed large masses suggesting lipomas in the neck. The patient underwent a two-stage lipectomy. This patient is the 13th case reported in japan since 1978, though over 200 cases have been reported since 1846 in europe, most of them from the Mediterranean (Kitano et al. ORL 1994;56:177 180; Kaku et al. Endocrinol. Diabetol. 1997;4:103-106).- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/11. Efficacy of lipectomy and liposuction in the treatment of multiple symmetric lipomatosis.BACKGROUND: Multiple symmetric lipomatosis (MSL) is a rare disease characterized by enlarging, symmetric, nonencapsulated, fat deposits mainly on the neck and upper trunk. Liposuction and lipectomy, although palliative, are the treatments of choice, especially indicated when vital structures are compromised. OBJECTIVE: Our purpose was to evaluate the efficacy and safety of liposuction and lipectomy in the treatment of MSL. methods: We have examined two patients diagnosed with MSL who presented with symptoms derived from the compression of vascular, nervous, and/or respiratory tract structures. One was treated with lipectomy and the other with liposuction. RESULTS: A rapid resolution of the clinical symptoms was achieved with both therapies. The patient who was treated with lipectomy suffered from a compression of the left brachial plexus by scar tissue as an adverse effect, requiring a second surgical procedure. Liposuction only provoked a mild autoinvolutive hematoma in the other case. No clinical recurrences were observed at 3 and 2 years of follow-up respectively. CONCLUSIONS: We consider both lipectomy and liposuction as safe and effective techniques for the treatment of MSL patients. Although liposuction is usually associated with less adverse effects than lipectomy, location of the lipomas must be carefully considered before choosing one technique over another.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/11. Ultrasound assisted liposuction for the palliative treatment of Madelung's disease: a case report.Benign Symmetric lipomatosis (Madelung's disease) is a rare disease, characterized by massive fatty deposits in the neck, the shoulders, and the upper extremities. The deformity is associated with chronic alcohol use, malignant tumors of the upper airways, neuropathy, diabetes mellitus, hyperlipidemia, and other metabolic disorders. Although the deformity is prone to recurrence, surgical removal via lipectomy or liposuction provides the only way of palliation. This paper describes the treatment of a 51-year-old man with a history of alcoholism and liver cirrhosis. He reported masses in his cervical and facial regions that had gradually enlarged over a period of 6 years. He also developed respiratory symptoms due to the fatty compression of his upper airways. Our experience with ultrasound-assisted liposuction for the palliative treatment of this disease is reported.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/11. Launois-Bensaude syndrome in a female with type 2 diabetes.BACKGROUND: The Launois-Bensaude syndrome (LBS) is a rare disease characterized by an accumulation of multiple lipomata with a preponderance at the thorax, abdomen, upper arms and the thighs. Frequently, the condition is associated with past or present alcohol abuse with no clear temporal coincidence between the onset and termination of lipomata growth and onset or termination of alcohol consumption. Due to the massive accumulation of adipose tissue, the patients frequently have features of the metabolic syndrome as hypertension, impaired fasting glucose or diabetes mellitus, hyperuricemia or hyperlipidemia. CASE REPORT: A 79-yr-old female observed an increase of fat mass especially at the upper arms, the thighs and the thorax in combination with a weight gain of 19 kg within 2 years without any changes in the nutrition habits. The unique features confirmed the diagnosis of LBS. Interestingly, she had diabetes mellitus, hypertension and hyperlipidemia before the manifestation of LBS and without any history of heavy alcohol consumption. Furthermore, the condition predominantly affects males. In her very case, treatment with sultanol, as successfully performed in some cases, could not be recommended due to her cardiac insufficiency. A surgical approach is of limited value due to the frequent relapses of the lipomata. CONCLUSIONS: Establishing the diagnosis of LBS is an important step for patients confronted with an inexplicable physical disfigurement that is not related to excessive nutrition. The disease is often not diagnosed because of its rareness, but its features are unique and easily to be distinguished from 'simple' truncal obesity.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/11. Multiple symmetric lipomatosis (Madelung's disease): report of two cases.Multiple symmetric lipomatosis (Madelung's disease) is a rare disease characterized by non-encapsulated adipose deposits in the neck, the superior part of the trunk and, very rarely, in the limbs. It is common in middle-aged Caucasian Mediterranean males but very rare in Chinese people. We describe two cases of Madelung's disease: both patients presented complaining of a development of a painless neck mass that was increasing in size. Panendoscopy was normal. Both underwent incisional biopsies that revealed benign adipose tissue characterizing Madelung's disease. We discuss the clinical presentation, associated morbidity, and treatments for Madelung's disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/11. Madelung syndrome (benign lipomatosis): clinical course and treatment.Madelung syndrome is a rare disease found predominantly in the Mediterranean area. It has a distinctive clinical appearance. Staged surgery is the treatment of choice, which produces substantial improvement in both functional and aesthetic appearance. Recurrences usually occur when it is impossible to resect the disease completely.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/11. Benign symmetric lipomatosis in a 73-year-old man: a case report with a brief review of the literature.Benign symmetric lipomatosis (BSL) is a rare disease which is characterized by symmetric diffuse deposition of mature fat tissue and considered to originate in brown fat. A case of benign symmetric lipomatosis in a 73-year old man is presented. He has been treated for alcoholic abuse in mental hospitals several times and referred to our hospital for evaluation and treatment of the subcutaneous tumor in the neck, bilateral supraclavicular areas, bilateral upper arms, anterior chest wall, back, and the abdomen. Resected tumors had ill-defined margin and smooth surface. Histologically, the tumor was composed of diffuse proliferation of mature fat tissues, focal myxoid change and spindle cell proliferation. A histopathological diagnosis of lipomatosis with focal spindle cell proliferation was made. Although several hypotheses of this disease have been postulated, the present case suggested that the etiology of BSL is closely related with alcoholic abuse-induced metabolic disorder and deteriorated function of adipocytes due to specific location of tumor.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/11. Madelung's disease.Madelung's disease is a rare disease of undetermined cause characterized by symmetric deposits of painless, diffuse, subcutaneous adipose tissue on the suboccipital area, the cheeks, the neck, the shoulders and the upper trunk. It is commonest in countries bordering the Mediterranean. The patients are usually middle-aged male alcoholics. Although lesions can initially mimic a head and neck malignancy, management is essentially symptomatic, with conservative removals done as indicated clinically or for cosmesis. Oriental reports about Madelung's disease are very rare. Here a case of Madelung's disease in a 59-year-old male is described. We describe his clinical course and review the literature.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/11. Madelung's disease (benign symmetric lipomatosis).Madelung's disease, benign symmetric lipomatosis, is a rare disease of undetermined cause characterized by symmetric deposits of diffuse adipose tissue on the cheeks, the neck, and the upper trunk. This article outlines our experience with four patients and presents a case report of a specifically remarkable affectation that agrees with previous data linking male alcoholics with this disease. The treatment in all cases was palliative surgical removal of excess fat from the neck and paracervical regions.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/11. Benign symmetric lipomatosis.We report a case of massive, benign symmetric lipomatosis associated with laryngeal compression. Current views on the aetiology and management of this rare disease are discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
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