ABSTRACT
We present a case of multiple symmetric lipomatosis Type I (Madelung's disease) with severe organic affection, hepatic cirrhosis, sever sensitive polyneuropathy and neuropathic ulceration at the left lower limb. A nephrotic syndrome developed in a larval form due to proliferative glomerulonephritis as the result of a metainfectious complications of the infection at the lower limb. We discuss the etiopathogenicity of the organic affection and we highlight the pathogenic links between the disease and its complications.
Subject(s)
Lipomatosis, Multiple Symmetrical/complications , Nephrotic Syndrome/complications , Biopsy , Glomerulonephritis/complications , Glomerulonephritis/etiology , Glomerulonephritis/pathology , Humans , Kidney/pathology , Leg Ulcer/complications , Liver/pathology , Liver Cirrhosis/complications , Liver Cirrhosis/pathology , Male , Middle Aged , Nephrotic Syndrome/pathology , Nervous System Diseases/complicationsABSTRACT
Multiple Symmetric Lipomatosis (MSL or Madelung's Disease) is a rare entity characterized by the presence of non-capsulated lipomas mainly located at the neck and upper part of the thorax and frequently associated to chronic hepatopathy, neuropathy and alcoholic habit. We review and update the clinical, etiopathogenic, diagnostic and therapeutical aspects of this rare entity. We stress the presence of neuropathy, a typical feature of the disease, which has also a predictive value.
