KOBBERLING-DUNNIGAN SYNDROME - A RARE CAUSE OF GENERALIZED MUSCULAR HYPERTROPHY

A 36-year-old woman presented with muscle hypertrophy (particularly of the carves) since puberty, occasional muscle cramps, a musculine habi tus, and a loss of subcutaneous fat on limbs and trunk sparing her fac e, neck, and vulva. Multiple lipomas were found on her trunk, and acan thosis nigricans on her neck. Laboratory testing revealed hyperlipidem ia and pathological glucose tolerance with hyperinsulinemia. Physical and laboratory findings are consistent with Kobberling-Dunnigan syndro me, a rare inherited form of lipoatrophy. The patient's mother had the same body habitus and insulin-dependent diabetes mellitus. These case s suggest that partial lipodystrophy also affects muscle and is a caus e of genuine muscular hypertrophy. (C) 1996 John Wiley & Sons, Inc.