Familial multiple lipomatosis

Familial multiple lipomatosis (FML) is a hereditary syndrome of multiple encapsulated lipomas which are found on the trunk and extremities, with relative sparing of the head and shoulders. 

Inheritance is frequently by autosomal dominant transmission, although cases with recessive inheritance have also been reported.

FML is a rare benign hereditary syndrome and an incidence of 0.002% has been reported. Men are affected twice as commonly as women . 

However, they can occur at an earlier age and have a more diffuse distribution, FML usually appear mostly in the third decade of life and affect several members of a family.

The lipomas associated with FML are usually painless and patients are not troubled by the disease.

Multiple, rubbery, nontender nodules which usually is located on the trunk and extremities. The lesions usually are mobile and nonfluctuant. Although tumors usually range in size from a few millimeters to 6 cm in diameter, lipomas as large as 25 cm have been reported. Spontaneous regression and malignant degeneration are rare. 

It is clinically distinct from multiple symmetric lipomatosis with which it is frequently confused in the dermatologic literature.

The disease is not associated with any abnormalities in lipid metabolism.

The main treatment consists of surgical excision of the lesions responsible for cosmetic and functional impairment. Other possible treatment techniques include liposuction and injection lipolysis with phosphatidyl choline

It is important to distinguish FML from other causes associated with subcutaneous lipomas such as Madelung's disease, Dercum`s disease, Bannayan-Zonana syndrome and Cowden syndrome.

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Article information

rID: 21874
Section: Pathology
Tag: cases
Synonyms or Alternate Spellings:
  • Familial multiple lipomatosis (FML)

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