Hereditary progressive nodular lipomatosis: a report and selective review of a new syndrome.
Multiple hereditary lipomatosis has been reported very rarely in the literature. The largest number of lipomas previously reported in a single patient was 160. We report on a 45-year-old man who began to experience multiple lipomas at about age 26. The lipomas multiplied until they severely disfigured his entire torso, arms, and upper legs. Both sides of his body were affected to approximately equal degrees. A family history revealed that the patient's father, two brothers, and two nephews have all experienced multiple subcutaneous lipomas, although to a lesser degree. In treating the patient, what appeared to be very large lipomas were in fact found to be subcutaneous clusters of hundreds of small encapsulated lipomas. Lipomas were excised using suction lipectomy to treat different involved areas on eleven separate occasions. A total of 8,475 cc was removed over a two-year period. A modified, slightly less blunt cannula was developed to aspirate the clustered lipomas individually.
Ersek
R A
RA
University of Texas Health Science Center, San Antonio.
Lele
E
E
Surak
G S
GS
Denton
D R
DR
McCue
K
K
D006801:Humans; D015187:Lipectomy / Q000379:methods; D008068:Lipomatosis / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D008297:Male; D008875:Middle Aged