Multiple Symmetric Lipomatosis: A Diagnostic Dilemma

Abstract

Introduction. Multiple symmetric lipomatosis, or Madelung's disease, is a rare condition which is characterized with large symmetrical accumulation of noncapsulated fat tissue in upper arms, neck, and shoulder areas. The disease etiology is unknown, with the highest incidence in the Mediterranean region. Case Presentation. Here, we present the case of Madelung's disease with symmetric fat distribution throughout the neck and history of alcoholism. The patient was treated from several diseases associated with alcoholism and hospitalized several times, but the diagnosis of Madelung's disease was omitted. The thyroid gland disease was excluded, while enlargement of the neck adipose tissue was attributed to obesity. Conclusions. This study points out possible diagnostic mistakes when a physician is not aware of a differentiation diagnosis of symmetrically enlarged neck masses, especially in geographic regions with high incidence of this disease.

An Unusual Case of Benign Symmetric Lipomatosis

“…Therefore, it is also known as Madelung's disease, Launois-Bensaude syndrome. In general, is characterized by the growth of nonencapsulated masses of adipose tissue [4]. In the following case the masses presented a thin capsule that was difficult to visualize in the ultrasound ( Figure 5).…”

“…Biopsy of fatty mass could be performed to confirm definitive diagnosis [4]. BSL could be complicated by somatic and autonomic neuropathy and by the infiltration of the adipose tissue at the mediastinal level.…”

Multiple Symmetrical Lipomatosis: Madelung’s Disease

“…Madelung's disease is characterized by diffuse, symmetric, painless, non-encapsulated, and irreversible growth lipomatosis [3]. The etiology of the disease is unknown but recent studies suggest that mitochondrial disorder of brown fat tissue or a defect in the adrenergic stimulated lipolysis, is involved Carvalho et al J Med Cases.…”

“…2016;7(5):178-180 [4,5]. Also, this disease is more common in the male gender (15:1 ratio), between 30 and 60 years old and in the Mediterranean population [3]. Chronic alcoholism history is present in 60-90% of the cases [2,6].…”

“…Madelung's disease phenotype may vary but most of the cases present with adipose tumefactions in the face, neck, occipital fossa and shoulder areas. Fat deposits around the cervical region form a "buffalo hump" and a "horse collar", while fat deposits around the parotid region may appear as "hamster cheek" [3,7]. Fat masses are symmetrically distributed, while distal arms and legs are spared [8].…”