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Associated Anomalies Observed in Two Siblings

MARIAN M. BRUBAKER, MD; NORMAN E. LEVAN, MD; P. J. COLLIPP, MD

Arch Dermatol. 1965;91(4):320-325.

Abstract
Congenital total lipodystrophy, a rare condition only recently recognized, has been observed in two siblings and followed for six years. The outstanding characteristics of the syndrome are complete absence of the panniculus with prominence of the muscles, hepatomegaly, accelerated linear growth and bony maturation, hyperlipemia, and disturbance of carbohydrate metabolism. insulin-resistant diabetes mellitus may develop after puberty. Striking dermatologic features, particularly hypertrichosis and hyperpigmentation, are also among the earliest signs. Both these patients presented congenital acanthosis nigricans, diagnosed in only one earlier case. Findings suggestive of acanthosis nigricans have been described in other reports. A diabetic trend in one of our patients has been observed recently. The relationship of acanthosis nigricans to congenital total lipodystrophy and to diabetes is uncertain at the present time.

Author Affiliations
LOS ANGELES

From the University of Southern California School of Medicine. Assistant Professor of Medicine (Dermatology), University of Southern California School of Medicine, Attending Physician in Dermatology, Childrens Hospital of Los Angeles (Dr. Brubaker); Professor of Medicine (Chairman, Section of Dermatology), University of Southern California School of Medicine (Dr. Levan); Instructor, Department of Pediatrics, University of Southern California School of Medicine; Division of Endocrinology, Childrens Hospital of Los Angeles (Dr. Collipp).

Footnotes
Accepted for publication Nov 25, 1964.

Reprint requests to 4529 Angeles Crest Hwy, La Canada, Calif 91011 (Dr. Brubaker).

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