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 subcutaneous tissue. These lipomas are most commonly found in the neck, shoulders, upper arms, and upper trunk, giving the appearance of a "buffalo hump" or "horse collar." Unlike typical lipomas, these growths are not encapsulated and can infiltrate surrounding tissues.

The condition is more prevalent in middle-aged men and is often associated with alcoholism. However, it can also occur in women and individuals without a history of alcohol abuse. The exact etiology of the disease is not well understood, but it is believed to involve mitochondrial dysfunction and alterations in lipid metabolism.

Pathophysiology

The pathophysiology of Launois–Bensaude adenolipomatosis is not completely understood. It is hypothesized that mitochondrial DNA mutations may play a role, leading to defects in oxidative phosphorylation and subsequent lipid accumulation. The condition is also associated with metabolic disturbances, including hyperlipidemia and insulin resistance.

Diagnosis

Diagnosis of Launois–Bensaude adenolipomatosis is primarily clinical, based on the characteristic distribution of lipomas. Imaging studies such as computed tomography (CT) or magnetic resonance imaging (MRI) can be used to assess the extent of lipomatous infiltration 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 primarily symptomatic and may include surgical removal of lipomas for cosmetic reasons or to alleviate discomfort. Liposuction is another option, although recurrence is common. Management of associated metabolic conditions, such as diabetes mellitus and hyperlipidemia, is also important.

Prognosis

The prognosis for individuals with Launois–Bensaude adenolipomatosis varies. While the condition is benign, it can lead to significant cosmetic and functional impairment. Early intervention and management of associated metabolic disorders can improve quality of life.

Also see

• Lipoma
• Liposarcoma
• Adipose tissue
• Mitochondrial diseases