Cutaneous Aspects of the XXYY Genotype: A Variant of Klinefelter's Syndrome

Five individuals with a variant of Klinefelter's (XXYY) syndrome are presented. The characteristics of the syndrome are discussed, including those signs of interest to dermatology. They include variations in body hair, lack of acne at adolescence, multiple cutaneous angiomas, acrocyanosis, and peripheral vascular disease producing the early onset of stasis dermatitis. The stasis change resembles that found in patients of normal genotype both clinically and histologically. The various effects of androgens and estrogens on peripheral vasculature are discussed.