Benign symmetric lipomatosis
|Benign symmetric lipomatosis|
|Synonyms||Benign symmetric lipomatosis of Launois–Bensaude, Madelung's disease, multiple symmetric lipomatosis, cephalothoracic lipodystrophy|
|A young man with benign symmetric lipomatosis (Madelung's disease) of unknown cause|
|Classification and external resources|
|ICD-10||E88.8 (ILDS E88.820)|
Benign symmetric lipomatosis is a skin condition characterized by extensive symmetric fat deposits in the head, neck, and shoulder girdle area. 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.
The cause of the disease remains unknown, but its incidence strongly correlates with alcohol abuse; abstinence from alcohol prevents disease progression. Defects in the adrenergic-stimulated lipolysis and accumulation of embryological brown fat have also been reported. Cosmetic disfigurement due to the fat deposition in the cervicothoracic region results in a "pseudoathletic appearance", resembling the Italian statue Warrior of Capestrano and carvings of Queen of Punt (Egypt). 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.
Art and culture
The appearance of people with the disease is depicted in:
- Carvings of Queen of Punt (Egypt), as noted above
- The Italian statue The Warrior of Capestrano, as noted above
- Donna Leon's crime novel, Beastly Things (2012), wherein the protagonist investigates the murder of a man who had the condition, which Brunetti learns has a high incidence in Italy
- 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. PMID 15164232. doi:10.1007/s00266-004-3123-5.
- Medappil Noushif; Vasu TA (July 2010). "Madelung's disease: A spot diagnosis". Indian J Plast Surg. 43 (2): 227–8. PMC . PMID 21217991. doi:10.4103/0970-0358.73472.
- Hasegawa T, Matsukura T, Ikeda S (June 2009). "Mesotherapy for Benign Symmetric Lipomatosis". Aesthetic Plast Surg. 34 (2): 153–6. PMID 19488808. doi:10.1007/s00266-009-9374-4.
- Leon, Donna (2012). Beastly Things (Commissario Brunetti, #21). Grove/Atlantic. p. 5 of Chapter 3. ISBN 0802120237.
|This cutaneous condition article is a stub. You can help Wikipedia by expanding it.|