Launois-Bensaude adenolipomatosis

Launois-Bensaude Adenolipomatosis

Launois-Bensaude adenolipomatosis, also known as multiple symmetric lipomatosis (MSL) or Madelung's disease, is a rare disorder characterized by the abnormal accumulation of adipose tissue, primarily in the upper body. This condition is named after the French physicians Paul Launois and Robert Bensaude, who first described it in the early 20th century.

Clinical Presentation

Patients with Launois-Bensaude adenolipomatosis typically present with symmetric, non-encapsulated lipomas located in the neck, shoulders, upper arms, and upper trunk. These lipomas can vary in size and may cause cosmetic concerns or physical discomfort. In some cases, the lipomas can compress surrounding structures, leading to symptoms such as difficulty breathing or swallowing.

Epidemiology

Launois-Bensaude adenolipomatosis is more common in males than females, with a male-to-female ratio of approximately 15:1. The condition is most frequently diagnosed in middle-aged adults, particularly those of Mediterranean or European descent. There is a strong association with alcohol consumption, and many patients have a history of chronic alcoholism.

Pathophysiology

The exact pathophysiology of Launois-Bensaude adenolipomatosis is not well understood. It is believed to involve a defect in the regulation of adipose tissue distribution and metabolism. Some studies suggest a mitochondrial dysfunction or a defect in the adrenergic regulation of lipolysis. Genetic factors may also play a role, although no specific genetic mutations have been consistently identified.

Diagnosis

Diagnosis of Launois-Bensaude adenolipomatosis is primarily clinical, based on the characteristic distribution of lipomas. Imaging studies such as ultrasound, computed tomography (CT), or magnetic resonance imaging (MRI) can be used to assess the extent of lipomatous involvement and to rule out other conditions. A biopsy may be performed to confirm the diagnosis and exclude malignancy.

Treatment

There is no definitive cure for Launois-Bensaude adenolipomatosis. Treatment is generally symptomatic and may include surgical removal of lipomas for cosmetic reasons or to relieve compression symptoms. Liposuction is another option for reducing the size of lipomas. Patients are advised to reduce alcohol consumption, as this may slow the progression of the disease.

Prognosis

The prognosis for patients with Launois-Bensaude adenolipomatosis varies. While the condition is benign, the progressive accumulation of lipomas can lead to significant physical and psychological morbidity. Early intervention and lifestyle modifications can help manage symptoms and improve quality of life.

Also see

• Lipoma
• Adipose tissue
• Madelung's disease
• Liposuction
• Alcoholism