Madelung disease, also known as Madelung-Launois-Bensaude syndrome or neck lipomatosis or multiple symmetric lipomatosis, is a rare benign entity clinically characterised 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 characterised 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.
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.
Blood lipid screen is usually normal 7.
Although the aetiology is unknown, approximately 60-90% of patients present a previous history of moderate to severe alcoholism or liver dysfunction.
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 deposits in a centripetal distribution. The swellings are non-tender on ultrasound.
Treatment and prognosis
Treatment centres 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.
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