madelung disease

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 , mainly in the posterior aspect. Limb involvement is usually proximal,, and around the shoulder and hip girdles.

Terminology

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.

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.

An association with alcohol abuse has been described .

Clinical presentation

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.

Blood lipid screen is usually normal .

Pathology

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 . Adipose cells usually have a benign microscopic appearance.

Distribution

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 .

Radiographic features

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 .

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.

Differential diagnosis

Possible considerations include

• obesity
• Cushing syndrome
• liposarcoma

Siehe auch:

• Lipomatose
• Madelung-Deformität
• Madelung dyschondro-osteosis

und weiter:

• fat redistribution syndrome