Introduction:
Diffuse overgrowth of mature adipose tissue
Types: multiple symmetric lipomatosis, infiltrating congenital lipomatosis of face, encephalocraniocutaneous lipomatosis, shoulder girdle lipomatosis, adipose dolorosa, pelvic lipomatosis, mediastinal lipomatosis
Multiple symmetric lipomatosis:
Brodie (1846)
Also known as Madelung disease, Launois-Bensaude syndrome
Almost exclusively middle-aged alcoholic men, common in Mediterraneans, may be AD
Painless progressive fat deposition in neck (donut-shaped collar of fat - lipoma annulare colli), upper trunk, arms, cheeks, and axilla.
Associated with sensorimotor neuropathy, tracheal compression,
CT and MR: infiltrative fatty masses in subcutaneous distribution, between deep muscles (commonly sternocleidomastoid, trapezius, paraspinal) into the mediastinum
Infiltrating congenital lipomatosis of face:
Slavin et al (1983)
Limited to cheek and face, causing facial asymmetry, parotid involvement, bone hypertrophy, macroglossia, cutaneous capillary blush, mucosal neuromas.
Encephalocraniocutaneous lipomatosis:
Haberland and Perou (1970)
Affects temporofrontal area unilaterally, cerebral tissues, leptomeningeal tissues, skull, eye, heart.
Associated with mental retardation, cerebral malformations, calcification, seizures
Shoulder girdle lipomatosis:
Enzi et al (1992)
Gradually progressive unilateral enlargement of shoulder and upper arm
Associated with neuromyopathy, compression of upper airway
Adiposa dolorosa:
Dercum (1892)
Multiple subcutaneous lipomas associated with pain and tenderness
Obese postmenopausal women almost always affected
May be AD, frequently have epilepsy and psychologic disease like emotional instability, depression, and dementia
Commonly seen in pelvic girdle, thighs, around hips and knees
Associated with motor weakness and fatigability.
Treatment options include injection of lidocaine and steroids.
Lipomatosis of nerve:
Also known as Neural fibrolipoma
References:
Murphey MD et al. Benign Musculoskeletal Lipomatous Lesions. RadioGraphics 2004;24:1433-1466
