Acne conglobata has been reported to be more common and more severe in
XYY chromosomal aberration than in normal subjects, whereas only exce
ptional cases have been observed in the more common XXY Klinefelter ka
ryotype. We report a 17-year-old male with both Klinefelter's syndrome
and acne conglobata, spreading from face to upper arms, trunk, buttoc
ks and upper legs. The serum level of testosterone was normal, whereas
luteinizing hormone and follicle-stimulating hormone were elevated. S
kin lesions responded well to a combined therapeutic regimen including
oral isotretinoin and prednisolone. An overview of severe acne in rel
ation to chromosomal abnormalities is given and diagnostic and therape
utic options discussed.