Benign symmetric lipomatosis
| Benign symmetric lipomatosis | |
|---|---|
|
A young man with benign symmetric lipomatosis (Madelung's disease) of idiopathic etiology | |
| Classification and external resources | |
| Specialty | endocrinology |
| ICD-10 | E88.8 (ILDS E88.820) |
| ICD-9-CM | 272.8 |
| OMIM | 151800 |
| MeSH | D008069 |
Benign symmetric lipomatosis (also known as Benign symmetric lipomatosis of Launois–Bensaude, Madelung's disease, multiple symmetric lipomatosis and cephalothoracic lipodystrophy) is a cutaneous condition characterized by extensive symmetric fat deposits in the head, neck, and shoulder girdle area.[1] The German surgeon Otto Wilhelm Madelung was the first to give a detailed description of the disorder. This condition is very rare with an estimated incidence rate of 1 in 25,000 and affects males up to 30 times more frequently than females.[2]
The cause of the disease remains unknown, but there is a strong correlation with alcohol abuse; abstinence from alcohol prevents disease progression. Defects in the adrenergic-stimulated lipolysis and accumulation of embryological brown fat has also been reported. Cosmetic disfigurement due to the fat deposition in the cervicothoracic region results in a 'pseudoathletic appearance'. This resembles the Italian statue Warrior of Capestrano and carvings of Queen of Punt (Egypt).[3] Traditionally the treatment is mainly surgical, consisting of the removal of the lipomas, although recent study has proposed liposuction and phosphatidylcholine injection as possible alternatives.[4]
See also
References
- ↑ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
- ↑ González-García R, Rodríguez-Campo FJ, Sastre-Pérez J, Muñoz-Guerra MF (2004). "Benign symmetric lipomatosis (Madelung's disease): case reports and current management". Aesthetic Plast Surg. 28 (2): 108–12; discussion 113. doi:10.1007/s00266-004-3123-5. PMID 15164232.
- ↑ Medappil Noushif; Vasu TA (July 2010). "Madelung's disease: A spot diagnosis". Indian J Plast Surg. 43 (2): 227–8. doi:10.4103/0970-0358.73472. PMC 3010793
. PMID 21217991. - ↑ Hasegawa T, Matsukura T, Ikeda S (June 2009). "Mesotherapy for Benign Symmetric Lipomatosis". Aesthetic Plast Surg. 34 (2): 153–6. doi:10.1007/s00266-009-9374-4. PMID 19488808.