Madelung disease

[Madelung] disease, also known as multiple symmetric lipomatosis, is characterized by benign, nonencapsulated fatty masses around the head and neck, the trunk and the upper extremities.1 Its prevalence is about 1:25 000 with men affected more frequently than women by a ratio of 20:1.1 The vast major...

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Bibliographic Details
Published in:Canadian Medical Association journal (CMAJ) 2013-01, Vol.185 (1), p.E79-E79
Main Authors: Lin, Fang-ying, Yang, Tsung-Lin
Format: Article
Language:English
Subjects:
Aged
Care and treatment
Connective tissue tumors
Diagnosis
Genetic disorders
Humans
Intervention
Lipectomy
Lipomatosis, Multiple Symmetrical - diagnosis
Lipomatosis, Multiple Symmetrical - pathology
Lipomatosis, Multiple Symmetrical - surgery
Male
Medical treatment
Mitochondria
Practice
Rare diseases
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Description
Summary:[Madelung] disease, also known as multiple symmetric lipomatosis, is characterized by benign, nonencapsulated fatty masses around the head and neck, the trunk and the upper extremities.1 Its prevalence is about 1:25 000 with men affected more frequently than women by a ratio of 20:1.1 The vast majority (90%) of people with Madelung disease have a history of alcoholism.1 Dysphagia and dyspnea may result from laryngeal or mediastinal involvement. The etiology of Madelung disease remains largely unknown.2 Most instances are sporadic, but a familial form characterized by maternally inherited mitochondrial gene mutation has been reported.3 Madelung disease may be associated with diabetes mellitus, hyperuricemia, hypothyroidism, liver disease or peripheral neuropathy. Its differential diagnosis includes solitary lip - oma, encapsulated lipoma, familial multiple lipomatosis and liposarcoma.1
ISSN:0820-3946
1488-2329
DOI:10.1503/cmaj.120191