JOHN D. BRUNZELL, M.D.; STEWART W. SHANKLE, M.D.; JOHN E. BETHUNE, M.D., F.A.C.P.
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Five siblings had congenital generalized lipodystrophy and systemic cystic angiomatosis. Manifestations of each disease and a suggested mechanism of cause related to rapid mobilization of fat stores are discussed. Evidence for autosomal recessive inheritance is presented. Serial studies of the five patients illustrate the progression of these diseases.
The simultaneous occurrence of these two diseases suggests that angiomatosis is a common, previously overlooked manifestation of congenital lipodystrophy or a new syndrome in a heterogeneous group of lipodystrophy disorders.
BRUNZELL JD, SHANKLE SW, BETHUNE JE. Congenital Generalized Lipodystrophy Accompanied by Cystic Angiomatosis. Ann Intern Med. ;69:501–516. doi: 10.7326/0003-4819-69-3-501
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Published: Ann Intern Med. 1968;69(3):501-516.
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Print ISSN: 0003-4819 | Online ISSN: 1539-3704
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