Multiple Hereditary Lipomatosis
Multiple hereditary lipomatosis is a hereditary skin and connective tissue condition characterized by the growth of unencapsulated masses of fat tissue (lipomas) symmetrically deposited around the neck, shoulders, or other sites around the body.
Lipomas are benign fat tissue tumors that can arise anywhere in the body. Thet are frequently encountered on the upper part of the body around the neck and shoulders. Most often they develop under skin, but sometimes can penetrate muscles. Lipomas can vary from a size of a pea to that of a soccer ball. The tumors are composed of fat enclosed in a thick, fibrous capsule. The fat (adipose) tissue of the tumor is often undistinguishable from normal fat.
Lipomas often produce a rounded mass that protrudes above the skin. On palpation, they feel smooth and compressible. The presence of multiple lipomas is known as lipomatosis. These lipomas rarely develop into cancers.
Up to several hundred of these lipomas can be present in lipomatosis. Familial cases have been described which seem to have a dominant inheritance pattern. Lipomatosis is thought to be caused by a defect in fat metabolism. Individuals with this condition have the appearance of obesity.
Lipomas are characterized by the absence of large blood vessels and nerve trunks, while lipomatosis generally has blood vessels and nerves.[4]
Besides surgical removal of lipomas, novel treatment of lipomatous tumors use antibodies derived from adipose tissue of a mammal donor. The methods are effective against both benign and malignant lipomatous tumors. The antibodies are used to decrease the size of lipomatous tumors, prevent the regrowth of removed tumors, and inhibit the proliferation of lipomatous cells.[3]
Canine lipomatosis has been described in Dachshunds. Dogs with this condition had progressively enlarging, pendulous skin folds involving the neck and trunk symmetrically. The skin was soft, with partial hair loss, and ulcerated.
Adapted from
1. Thomas J Zuber and E. J Mayeaux. Atlas of Primary Care Procedures
2. Montgomery/AAro. Clinical Pathology of Soft-Tissue Tumors
3. United States Patent Application 20070048323. Kind Code A1. Rubin; J. Peter March 1, 2007. Antibody treatment of lipomatous tumors
4. Peter J. Ihrke, Emily J. Walder, Verena K. Affolter, Thelma Lee. Skin Diseases of the Dog and Cat
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