Non-insulin-dependent diabetes mellitus with type I multiple symmetrical lipomatosis: a case report

Type I multiple symmetrical lipomatosis (MSL; Madelung's disease) is characterized by lipomas in the nape of the neck and the supraclavicular and deltoid regions, resulting in a bull-necked appearance (Madelung's collar). It is most common in alcoholic men between 35 and 50 years of age. Type I MSL has been reportedly associated with hyperinsulinemia, but its association with diabetes mellitus is rarely discussed. We describe a case of non-insulin-dependent diabetes mellitus (NIDDM) associated with type I MSL. A 47-year-old alcoholic man presented with a seven-year history of hyperglycemia and progressive neck swelling with dysphagia for one year. Physical examination showed diffuse and symmetrical swelling of the bilateral posterior aspects of the neck. Biochemistry profiles revealed elevated concentrations of fasting serum glucose (276 +/- 16 mg/dl), triglycerides (358 +/- 79 mg/dl) and total cholesterol (323 +/- 28 mg/dl). Endocrinologic studies showed normal thyroid function. Neck sonography revealed diffuse thickening and swelling of the fatty structures of both sides of the neck. Normal sonography showed no fatty deposition in the liver. Maxillary and neck computerized tomography revealed diffuse fat accumulation in the submental and posterior neck regions, with no extension to the superior mediastinum. Fine needle aspiration cytology of the neck masses showed only fat cells. The patient received an oral hypoglycemic agent (glibenclamide 5 mg bid) for blood glucose control and lovastatin (20 mg before bed-time) for hyperlipidemia, and ceased drinking alcohol. The neck swelling resolved markedly after 15 months of medical treatment. This suggests that, in addition to the cessation of alcohol consumption, the reduction of blood glucose and lipid concentrations by medication may also assist in resolving the accumulated fat of type I MSL in patients with NIDDM.