Multiple symmetric lipomatosis (MSL), also known as benign symmetric lipomatosis (BSL), Madelung’s disease, or Lanois-Bensaude syndrome, is a rare abnormal fat metabolism disease.
The pathogenesis is not clear, but long-term alcohol abuse is considered to be the main cause. Large amounts of ethanol impair liver function, and lipodystrophy caused by hepatic dysfunction results in abnormal fat deposition, eventually leading to the disease. The disease may be associated with mitochondrial gene mutations and abnormal brown adipocytes.
Signs and Symptoms
According to the clinical manifestations, the disease may be divided into two types.
Type I occurs mainly in males with a long-term history of alcohol abuse. Lipomas are diffusely distributed in the neck, upper back, shoulders, chest, abdomen, and proximal limbs, mostly neck. The disease progresses slowly. Large lipomas can compress the esophagus and cervical spine, resulting in difficulty in breathing, swallowing, and neck mobility. Invasion of the tongue can cause megaglossia, affecting tongue movement and swallowing. The involvement of the parotid glands can cause facial deformities.
Figure 1 multiple symmetric lipomatosis
Figure 2 multiple symmetric lipomatosis
Type Ⅱ can occur in both males and females. Fat is diffusely distributed throughout the body, resembling simple obesity. However, the cellulite will not decrease or subside even if there are alcohol cessation, low-fat diet, or weight loss. The enlargement of adipose tissue is mainly due to proliferation of adipocytes in this disease, while simple obesity is a result of enlarged volume of fat cells without increased count.
The disease can occur in children as well, and the disease may be congenital or accompanied by mental retardation. The disease in children is suggested to be type III.
There are diffuse, symmetrical fat deposition and thickening on the neck, shoulders, back, upper chest wall, subcutaneous areas, platysma muscle, sternocleidomastoid muscle, trapezius muscle, or areas between muscles and blood vessels. There is no obvious boundary with normal tissues, and there is no obvious capsule. There may be linear or reticular fibrous septa, calcification, or ossification in the fat mass.
The mass is composed of mature adipocytes, and fibers, blood vessels, and mild inflammatory cell infiltration can be seen.
If there are diffuse, symmetrical, painless, and irreversible fat deposition, the disease can be diagnosed.
The surgery includes open surgical resection and liposuction.
Conservative treatment includes mitochondrial cocktail, extracorporeal acoustic wave therapy, laser lipolysis, and high intensity focused ultrasound.
This disease is benign, with a good prognosis, with a recurrence rate of 20% - 25%. Recurrences are mostly in patients with alcohol abuse after treatment. Complete alcohol cessation after surgical excision can help to prevent recurrence.