Do you know this syndrome? Type 2 benign symmetric lipomatosis (Launois-Bensaude)

Abstract

A 57-year-old female showed bulky, loose tumors, which progressively spread to her arms, anterior chest, and back. She reported dysphagia and dyspnea after mild exertion. She denied alcohol consumption. CT scan of her chest showed no internal lesions. Benign symmetric lipomatosis is a rare syndrome, clinically described as multiple nonencapsulated lipomas of various sizes and symmetrical distribution. This syndrome has three known phenotypes; in type 2 (Launois-Bensaude syndrome), lesions occur primarily on the shoulders, upper arms, and chest, and is unrelated to alcoholism. It causes aesthetic deformities and might block the upper airways. Mediastinal invasion might occur as well.

Madelung’s disease – progressive, excessive, and symmetrical deposition of adipose tissue in the subcutaneous layer: case report and literature review

“… 7 The results of a long-term study, in which 7 patients were observed over a period of 12 years, confirm the hypothesis that lipomas formed in the course of the disease are the result of mitochondrial disorders of BAT. 12 , 22 Other authors also indicate that disorders of proliferation and differentiation of human BAT cells underlie the described disease. 1 , 4 , 10 …”

“…Apart from these disorders, sensory, motor, and autonomic polyneuropathy is additionally observed in approximately 85% of patients with Madelung’s disease. 9 , 12 …”

“… 1 , 2 , 4 , 9 It is recognized that the disease may be inherited in an autosomal dominant manner with variable mitochondrial DNA penetration. 3 , 8 , 10 , 12 …”

“…The disease should be differentiated from morbid obesity, Cushing’s syndrome, salivary gland disease, lymphoma, Froelich’s syndrome, angiolipomatosis, myxoid liposarcoma, encapsulated lipomas, neurofibromatosis, and lipomatosis in patients infected with HIV. 4 , 12 The disease progresses rapidly in the first few years, after which the progression usually slows down or the size of the created adipose tissue mass stabilizes. 3 , 8 …”

“…Madelung’s disease affects men more often – the ratio of men to women varies from 15/1 to 30/1 – and is usually diagnosed between 30 and 70 years of age. 1 , 4 , 12 – 14 Most cases of the disease are reported in the Mediterranean region – the incidence in Italy has been reported to be as high as 1 in 25,000 men. 1 , 4 , 5 , 9 , 15 In contrast, in the Asian population, this disease is extremely rare.…”