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Congenital Generalized Lipodystrophy Accompanied by Cystic Angiomatosis
- JOHN D. BRUNZELL, M.D.;
- STEWART W. SHANKLE, M.D.; and
- JOHN E. BETHUNE, M.D., F.A.C.P.
- Requests for reprints should be addressed to John E. Bethune, M.D., University of Southern California School of Medicine, 2025 Zonal Ave., Los Angeles, Calif. 90033.
SUMMARY
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.
Article and Author Information
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From the Department of Medicine, Los Angeles County-University of Southern California Medical Center, Los Angeles, Calif.
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This study was supported in part by grant (FR-43), The General Clinical Research Centers; and by grant AM-06148, National Institutes of Health; Bethesda, Md.
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- Received March 25, 1968.
- Accepted May 1, 1968.
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