Benign symmetric lipomatosis (Madelung's disease): case reports and current management

Abstract

Benign symmetrical lipomatosis (BSL) is a rare disorder characterized by the presence of multiple, symmetric, nonencapsulated fat masses in the face, neck, and other areas. Typically, this entity has been related to the presence of three anterior bulges in the neck. The disorder was first described by Brodie in 1846. After that, Madelung in 1888 and Launois and Bensaude in 1898 characterized the disease. There are multiple synonyms for this disorder, such as Madelung's disease, Launois-Bensaude syndrome, and multiple symmetrical lipomatosis. Benign symmetric lipomatosis is usually described in adults from 30 to 60 years old, with an incidence of about 1 in 25,000 and a male-to-female ratio of 15:1 to 30:1. Most cases have no hereditary pattern. More than 90% of the patients have associated alcoholism. The etiology of benign symmetric lipomatosis remains unknown, but an abnormal lipogenesis induced by catecholamines has been observed. The transformation of BSL to a malignant tumor is extremely rare. In the current report, the authors describe two cases of benign symmetric lipomatosis treated in their department and a review of the literature.