Madelung disease

Madelung disease is a rare benign entity (lipomatosis) 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.

It should not be confused with the Madelung deformity of the wrist. 

Epidemiology

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.  

Pathology

Although the etiology is unknown, approximately 60-90% of patients present a previous history of moderate to severe alcoholism.

Distribution

It is mainly deposited along the anterior or posterior subcutaneous tissue of the neck, deep under the sternocleidomastoid and trapezius muscles, posterior cervical triangle, and around the salivary glands ⁴.

Treatment and prognosis

Treatment options include surgical resection (lipossuction or conventional surgery), indicated when there is evidence of aerodigestive compression and presence of aesthetic deformities. Recurrence is frequently observed. Abstinence of alcohol is advised if there is a relevant history.

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.

See also

Unrelated entities

• Madelung deformity: epiphyseal growth plate disturbance characterised by dorsal and radial bowing of the radius
• Madelung dyschondrosteosis: dysplasia associated with a Madelung deformity