Benign symmetrical lipomatosis or Madelung's disease was first described by Sir Benjamin Brodie in 1846, but later Otto Madelung reported the first series of 33 patients in 1888. In the following years, Launois and Bensaude presented a series of 65 patients. Enzi, in 1977, coined the descriptive term benign symmetric lipomatosis (LSB), since only 200 cases have been reported in the literature.
The term "lipomatosis" was generalized to refer to a disorder characterized by multiple lipomas, unencapsulated affecting several regions. In this definition, of which there are several classifications (Table 1), family entities include autosomal dominant and sporadic.
Table 1. Classification of lipomatosis.
• Benign symmetric lipomatosis (Madelung's disease)
- Type I (Madelung 's collar)
- Type II
• abdominal mediastinal lipomatosis
• pelvic lipomatosis
• epidural lipomatosis
• Adiposis dolorosa (Dercum Syndrome)
Enzi LSM classified into two types according lipomatous tissue distribution: one in which the fatty tumors are well demarcated and another aspect in which the diffuse lipomatous tissue extends giving the appearance of simple obesity. In type I tissue maintains the appearance of lipomatous masses arranged symmetrically in the upper body, whose growth appears to be slow and often affects deep locations. Adipose tissue is often atrophic involved. You can quickly spread to the chest, abdomen, arms and legs. At this stage, the only difference between type II by the presence of symmetric masses in the upper body and involvement of extremities (forearms and legs). In the LSM type II, despite the rapid and extensive growth into the chest, abdomen and extremities are not affected deeply appreciated. He also described the involvement of hands, feet and even language.
Benign symmetric lipomatosis, also called Madelung syndrome or Launois-Bensaude syndrome, is a rare entity of unknown etiology that primarily affects middle-aged men (30-60 years) in the Mediterranean area is often associated with alcohol abuse or liver disease
to chronic. It has also been associated with diabetes mellitus, hyperuricemia, chronic liver disease, hypertension, dyslipidemia, renal tubular acidosis, glucose intolerance, macrocytic anemia, polyneuropathy and HIV type I patients on antiretroviral therapy
The molecular basis of the genetic defect that causes this disease is unknown, although there are currently two theories to explain its pathogenesis: the first described by Enzi et al. in a series of 10 cases of LSB which advocates a defective lipolytic response to catecholamines appears to be related to mitochondrial alterations
and the second calls for an alteration of adrenergic stimulation of lipolysis caused by the autonomy of adipocytes
(resistant to such stimulation). SourceSome not so nice photos...