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Madelung's disease and alcoholic liver disorder

โœ Scribed by Mei-Ju Ko; Hsien-Ching Chiu


Book ID
102241309
Publisher
John Wiley and Sons
Year
2009
Tongue
English
Weight
292 KB
Volume
51
Category
Article
ISSN
0270-9139

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โœฆ Synopsis


A 53-year-old man presented with a 4-month history of multiple subcutaneous tumors on his head, neck, and upper trunk. He drank more than 1 L of rice wine per day, beginning at age 16. Multiple ill-defined, variously sized tumors were noted on the scalp, neck, shoulders, and upper trunk (Fig. 1). The largest, 28 cm in length, was seen on the posterior neck and upper back (Fig. 2). These tumors were elastic firm on palpation and showed no symptoms and signs of inflammation. Laboratory analyses revealed elevated aspartate aminotransferase, 83 U/L (ฯฝ31); elevated alanine aminotransferase, 43 U/L (ฯฝ31); and elevated serum bilirubin level, 1.8 mg/dL (0.2-1.0). The complete blood count with differential, blood glucose, and renal function tests were all unremarkable. Serologic tests ruled out viral hepatitis. Abdominal ultrasonography revealed fatty liver but no other abnormalities. Alcoholic liver disease was diagnosed. Skin biopsy and computed tomography of the tumors showed prominent fatty tissue (Fig. 3).

These symmetrically distributed fatty tumors on the back, suboccipital region, and proximal extremities, with a characteristic "horse-collar" appearance, are typical of Madelung disease (benign symmetric lipomatosis), a rare disorder that usually affects middle-aged alcoholic men. The differential diagnosis includes Cushing's syndrome, familial multiple lipomatosis, Dercum's disease, and congenital lipomatosis. Madelung disease predominantly affects men between the ages of 30 and 60 years. 1 The diagnosis is primarily dependent on clinical history and characteristic appearance. The masses are nonencapsulated, infiltrative, hypervascular. They can eventually reach very large sizes, diminish the range of motion of the neck and upper extremities, and even result in dysphagia or dys-


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