Partial face-sparing lipodystrophy (Köbberling-Dunnigan syndrome): report of a sporadic case.

A sporadic case is presented of a 20-year-old female with partial face-sparing lipodystrophy, also known as the Köbberling-Dunnigan syndrome with the Type 1 clinical phenotype. This disorder affects the limbs and spares the face. The trunk is spared in the Type 1 phenotype but is affected in the Type 2 phenotype. The patient had the associated features of this syndrome including the hyperinsulinaemia, acanthosis nigricans and mildly raised triglycerides, but not diabetes mellitus.