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Köbberling-Dunnigan syndrome: A rare cause of generalized muscular hypertrophy

✍ Scribed by Susanne Wildermuth; Stephanie Spranger; Matthias Spranger; Friedhelm Raue; Hans-Michael Meinck


Publisher
John Wiley and Sons
Year
1996
Tongue
English
Weight
521 KB
Volume
19
Category
Article
ISSN
0148-639X

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✦ Synopsis


A 36-year-old woman presented with muscle hypertrophy (particularly of the calves) since puberty, occasional muscle cramps, a musculine habitus, and a loss of subcutaneous fat on limbs and trunk sparing her face, neck, and vulva. Multiple lipomas were found on her trunk, and acanthosis nigricans on her neck. Laboratory testing revealed hyperlipidemia and pathological glucose tolerance with hyperinsulinemia. Physical and laboratory findings are consistent with Kobberling-Dunnigan syndrome, a rare inherited form of lipoatrophy. The patient's mother had the same body habitus and insulindependent diabetes mellitus. These cases suggest that partial lipodystrophy also affects muscle and is a cause of genuine muscular hypertrophy. 0 1996