Madelung Disease Affecting Scrotal Region.

Madelung disease is an uncommon disease characterized by large subcutaneous adipose masses. Mediterranean countries show higher disease incidence. We review the current concepts concerning this pathology, including etiology, diagnosis, and treatment. Madelung disease usually affects the upper half of the body, but we describe a patient with scrotal lipomas. As this location is rare, we review the cases found in literature, as well as the specific nuances regarding local symptoms and treatment.

Radiology Case Of The Month: Mental Disturbance for 4 days.

A 56 year old African-American man presented to the emergency department with dyspnea and dysphagia with drooling. On his initial evaluation, disproportionate obesity of the face, neck and shoulders were noted. The patient's history was significant for obstructive sleep apnea, end-stage renal disease, alcoholic liver disease, pulmonary hypertension and alcoholic cardiomyopathy. He had multi-decade history of heavy alcohol abuse, but quit drinking two years previously.

Exceptional manifestation of Madelung's disease after liver transplantation.

Unlike simple obesity, Madelung's disease (MD) is a rare disease characterized by symmetric accumulation of massive adipose tissue on the neck, the superior part of the trunk and limbs, leading to a pathognomonic cosmetic deformity. Here, we report an extremely rare case of MD associated with bilateral gynecomastia in a 61-year-old man, with a history of recent liver transplantation for alcoholic liver disease (ALD).

The endocrine and metabolic evaluation of benign symmetrical lipomatosis: a case report and literature review.

OBJECTIVE: Benign symmetrical lipomatosis (BSL) is a rare disease characterized by the presence of multiple, symmetric and nonencapsulated fat masses. Alcoholism is observed in nearly 90% patients. However the etiology of this disease is still unclarified. BSL is very rare in Chinese people. Herein we described the endocrine and metabolic status of a patient with typical BSL. We also discussed the clinical manifestation, etiology, diagnosis and treatment for BSL. RESULTS: Hyperuricemia and abnormal liver enzyme levels were observed in this case. However insulin sensitivity and function of the thyroid, adrenal glands and pituitary were all normal. The insulin sensitivity was assessed by the hyperinsulinemic euglycemic clam. CONCLUSIONS: Although an association had been found between BSL and some endocrinological or metabolic disorders including abnormal glucose tolerance, excessive secretion of insulin, hyperuricemia, and so on, they were not specific for BSL. It is necessary to develop into the pathogenesis of BSL further.

[About 10 cases of Launois-Bensaude disease]. / La maladie de Launois-Bensaude : description, prise en charge. A propos de dix patients opérés.

Launois-Bensaude syndrome is a rare pathology. The presence of multiple, symmetric, non encapsulated lipomatous masses in the face, neck, upper arms and upper trunk is typical. Men are especially affected between age of 35 and 50. The disease is frequently associated with alcoholism, hepatopathy, glucose intolerance, hyperuricemia, and malignant tumors of the upper airways, requiring thorough clinical evaluation of all patients. Cosmetic deformity and compression lead patients to seek treatment. Therapy is difficult. Dietary treatment and weight loss are of limited value in the management of this pathology. Surgery is the only effective treatment. The surgeon must make sure that the patient has ceased his alcohol abuse before performing this treatment. Conventional surgery or liposuction are used to restore a normal social life to these patients, deformed by their disease. This surgical management has a severe tendency to haemorrhage, dissection is complicated by infiltrations of neighbouring tissue and is frequently followed by recurrence, mainly after liposuction. Nevertheless, based on a review of ten cases, good results of surgical removal must prompt to operate them on.

Madelung disease: A case report.

RATIONALE: Madelung disease (MD), a rarely reported disease, also known as benign symmetric lipomatosis, a disorder resulting from alcoholic abuse. It's largely under-recognized and under-reported, possibly because of unawareness of the condition by physicians. PATIENT CONCERNS: A 45-year-old Chinese man presented with intermittent fatigue and abdominal distension and progressive bilateral breast enlargement. He has been a heavy drinker for ten years before onset of the disease with an average daily alcohol intake of more than 120 g/day. DIAGNOSIS: Due to the patient's symptoms, laboratory test results, radiographic findings, he was diagnosed with MD. INTERVENTIONS: We treated him with abstinence from alcohol and supportive therapy. OUTCOMES: The patient is now in stable condition, with improvement in symptoms during follow-up. LESSONS: Doctors, confronted with progressive bilateral breast enlargement in a patient with alcoholic liver disease, should be aware of the underreported MD. Recognition of this syndrome could help doctors establish diagnosis and emphasize the importance of alcohol abstinence as the mainstay of management.

Madelung's disease and acute alcoholic hepatitis: case report and review of literature.

Madelung's disease is a rare condition characterized by symmetric growth of fatty tumors (lipomas) around the neck, shoulders, upper arms, and trunk. It often affects men with a history of alcohol abuse. Here we report a review of the literature about this disease together with the description of a patient affected by Madelung's disease and acute alcoholic hepatitis.

Induction of benign symmetric lipomatosis (Launois-Bensaude syndrome) in a female patient after liver transplantation.

Benign symmetric lipomatosis, also known as Madelung's disease or Launois-Bensaude syndrome, is a rare disease, the etiology of which is still unknown. The presence of multiple, symmetric, nonencapsulated lipomatous masses in the face, neck, upper arms and upper trunk is typical. Until now many causes have been discussed among which liver dysfunctions are described frequently. In up to 90% of patients, alcoholism is observed. In our case the Launois-Bensaude syndrome developed after liver transplantation in a 49-year-old female patient suffering from decompensated cirrhosis (Child-Pugh C score: 12 points). Shortly after the transplantation a slow progress in tissue-building appeared on both upper arms, cervical areas as well as in the face. During postsurgical prednisolone therapy, a massive increase in fluid in the tissue developed, which led to a discontinuation of this therapy regimen. In the further course there was an increase in weight of 20 kg. As far as we know, this case is the first description of the induction of a Launois-Bensaude syndrome following liver transplantation.

Multiple symmetric lipomatosis - a reflection of new concepts about obesity.

Multiple symmetric lipomatosis (MSL) is characterized by subcutaneous accumulation of nonencapsulated adipose tissue. In type 2 MSL accumulation occurs on proximal limbs, upper back and hips. This sometimes unrecognized disease is similar to an exaggerated female fat distribution and can be confused with simple obesity. Obesity is a heterogeneous disorder and we suppose that type 2 MSL might have a place on the edge of the obesity spectrum. Several contemporary concepts about adipose tissue could be recognized in the model of MSL. Changes in fat distribution among different depots of adipose tissue in obesity have emerged as origin of its metabolic complications. Decreased insulin resistance and raised adiponectin have been found in MSL just as in some other conditions with accumulation of the subcutaneous adipose tissue (SAT). In that context, MSL may present as a model for possible favourable metabolic impact of SAT depots. Adipogenesis in MSL is not a consequence of energy excess but it is an active hyperplastic proliferation of SAT. This kind of behaviour of some adipocytes in several subcutaneous areas in MSL suggests that the energy unrelated adipogenesis could contribute to the expansion of at least a part of SAT depot in obesity in general. Contrary to current concept that the signals for adipogenesis are dependent only on the energy equation, allowing this additional mechanism would imply a new approach to issues of obesity, foremost to differentiate its particular types for which these concepts may be relevant.

Madelung's Disease: A Rare Cause of Dyspnea

No disponible

Launois-Bensaude syndrome and Bureau-Barrière syndrome in a psoriatic patient: successful treatment with carbamazepine.

Authors report a 41-year-old male patient with multiple symmetric lipomatosis, who also had psoriasis. In addition he suffered from peripheral neuropathy, acro-osteolysis and malum perforans pedis fulfilling the criteria of Bureau-Barrière syndrome. Considering the severe peripheral neuropathy and the preliminary report on the efficacy of carbamazepine in psoriasis, a carbamazepine monotherapy was initiated for both indications. The psoriatic skin symptoms cleared within 6 weeks. The patient has continued this medication for 8 months without side effects. During that time his skin remained symptom free and the neuropathy has improved.

Mitochondrial respiratory function in multiple symmetrical lipomatosis: report of two cases.

Madelung's disease is a rare benign disorder characterized by symmetrical deposition of adipose tissue on the neck and shoulders. The cause of Madelung's disease is obscure and may be associated with mitochondrial dysfunction. In this communication, we report two cases encountered at Cardinal Tien Hospital during 1992. Besides physical check-up and laboratory examination, adipose tissue and muscle biopsies from the biceps brachii were performed. As lipid metabolism is closely related to mitochondrial respiration, biochemical studies may be of great value in clarifying the pathogenetic mechanism of Madelung's disease. We thus determined the activities of mitochondrial respiratory enzymes (NADH cytochrome c reductase, succinate cytochrome c reductase, cytochrome c oxidase) in muscle and blood cells and revealed that the electron transport functions were all elevated. Molecular analysis of mitochondrial DNA from the muscle and blood cells of both patients failed to find large-scale deletion or point mutations.

[Symmetric multiple lipomatosis. A case report with an unusual onset]. / Lipomatosi multipla simmetrica. Descrizione di un caso clinico con inusuale insorgenza.

The authors report a case of symmetric multiple lipomatosis in a young woman with chronic liver disease caused by previous alcohol abuse whose onset occurred three years after the suspension of the toxic agent. The introduction includes a review of the latest literature, focusing in particular on the most probable etiopathogenetic causes, the most common clinical presentations and therapeutics choices. After a description of this particular case, the authors discuss the unusual presentation of the syndrome caused by the rapid onset of lipid accumulation a long time after the suspension of alcohol abuse.

Madelung's disease: an uncommon disorder of unknown aetiology?

A case of Madelung's disease (benign multiple symmetrical lipomatosis, Launois-Bensaude syndrome) is described. The characteristic clinical features, associations with chronic alcoholism and a review of the current literature is reported.

[Clinical aspects and therapy of benign symmetrical lipomatosis--Madelung disease]. / Klinik und Therapie der benignen symmetrischen Lipomatose--Morbus Madelung.

The clinical picture, diagnosis, therapy, and theories on the etiology of benign symmetric lipomatosis (Madelung disease) are outlined. Personal experience from 62 operations on 28 patients is compared to the literature. Up to now, there is only palliative surgical treatment for this sporadically appearing disease of unknown etiology. Liposuction--a less traumatic treatment--has considerably facilitated the treatment of the circumscribed fatty hyperplasias. The surgical characteristics of different anatomical regions are pointed out.