Cutis marmorata telangiectatica congenita and extensive mongolian spots: type 5 phacomatosis pigmentovascularis

Two patients with an unusual association of extensive cutis marmorata telangiectatica congenita and aberrant mongolian spots are reported. We believe that such association is best explained as a phenomenon of non-allelic twin spotting. We found only one previous similar case in a textbook, and we support the classification of this condition as phacomatosis pigmentovascularis type V.