Madelung disease, also known as Madelung-Launois-Bensaude syndrome or neck lipomatosis or multiple symmetric lipomatosis, is a rare benign entity clinically characterized by the presence of multiple and symmetric, non-encapsulated masses of fatty tissue, usually involving the neck and the upper region of the trunk; limb involvement is usually proximal, around the shoulder and hip girdles.
It should not be confused with the Madelung deformity of the wrist, which is an epiphyseal growth plate disturbance characterized by dorsal and radial bowing of the radius, or Madelung dyschondrosteosis, a dysplasia associated with the Madelung deformity.
Furthermore, this is a distinct entity from the similarly named familial multiple lipomatosis with which it is frequently confused in the dermatologic literature.
Madelung disease is most commonly seen in the Mediterranean population with a male to female ratio of 15:1. The commonest age of onset is between the third and fifth decades.
An association with alcohol abuse has been described 8.
Painless, symmetric soft tissue masses of the shoulders, upper arms, thighs, and neck, resulting in a pseudoathletic appearance, mimicking the body type of an athlete. Masses may compress aero-digestive structures resulting in dysphasia, dyspnea or may limit neck movements 8.
Blood lipid screen is usually normal 7.
Although the etiology is unknown, approximately 60-90% of patients present a previous history of moderate to severe alcoholism or liver dysfunction.
The fatty deposits are typically unencapsulated, with non-destructive infiltration and displacement of surrounding structures 9. Adipose cells usually have a benign microscopic appearance.
It is mainly deposited along the anterior or posterior subcutaneous tissues of the neck, deep under the sternocleidomastoid and trapezius muscles, posterior cervical triangle, and around the salivary glands 4.
Symmetric lobulated subcutaneous fatty deposition in a centripetal distribution. The swollen regions are non-tender on ultrasound.
Treatment and prognosis
Treatment centers around surgical removal, including liposuction, lipectomy or injection lipolysis. Lesion removal is primarily for aesthetic reasons, although occasionally secondary aerodigestive compression mandates intervention. Recurrence is frequently observed. Abstinence from alcohol is advised if there is a relevant history. Spontaneous resolution is not seen 7.
History and etymology
Its first description was in 1846 by Benjamin Brodie. The classical horse collar pattern of cervical lipomas distribution was described by Otto W Madelung in 1888.
Launois and Bensaude concluded the description of the syndrome in 1898, naming it as multiple symmetrical adenolipomatosis.
Possible considerations include
- 1. Farias TP, Dias FL, Freitas EQ, et al. Tratamento cirúrgico da doença de Madelung: relato de caso. Rev Bras Cir Cabeça Pescoço. 2003;32:23-6.
- 2. Adamo C, Vescio G, Battaglia M et-al. Madelung's disease: case report and discussion of treatment options. Ann Plast Surg. 2001;46 (1): 43-5. Ann Plast Surg (link) - Pubmed citation
- 3. Ampollini L, Carbognani P. Images in clinical medicine. Madelung's disease. N. Engl. J. Med. 2011;364 (5): 465. doi:10.1056/NEJMicm1003531 - Pubmed citation
- 4. Zhang XY, Li NY, Xiao WL. Madelung disease: manifestations of CT and MR imaging. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2008;105 (5): e57-64. doi:10.1016/j.tripleo.2008.01.004 - Pubmed citation
- 5. Ahuja AT, King AD, Chan ES et-al. Madelung disease: distribution of cervical fat and preoperative findings at sonography, MR, and CT. AJNR Am J Neuroradiol. 1998;19 (4): 707-10. AJNR Am J Neuroradiol (abstract) - Pubmed citation
- 6. Landis MS, Etemad-Rezai R, Shetty K et-al. Case 143: Madelung disease. Radiology. 2009;250 (3): 951-4. Radiology (full text) - doi:10.1148/radiol.2503071024 - Pubmed citation
- 7. Dupont J, Van der Schueren B, Mertens A. Launois-Bensaude syndrome after liver tranplantation. BMJ 2018;360:k1051.
- 8. Nisi, Giuseppe, Sisti, Andrea. Madelung’s Disease. (2016) The New England journal of medicine. 374 (6): 572. doi:10.1056/NEJMicm1503861 - Pubmed
- 9. Paul A. Levine, Darius Kohan, Philip J. Miller, Stephen G. Rothstein, David Kaufman. Madelung's Disease: Case Reports and Literature Review:. (1993) Otolaryngology—Head and Neck Surgery.