Cutis marmorata telangiectatica congenita: report of 22 cases

Twenty-two cases of cutis marmorata telangiectatica congenita were evaluated during an 8-year period. All but two patients were examined in the first year of life; 14 of the 22 (64%) were female infants. Four patients had focal cutaneous atrophy associated with the reticulated vascular pattern, and eight had ulcerations of involved skin. Six (27%) had additional anomalies. Of these, three patients had a nevus flammeus, and one had congenital generalized fibromatosis and hemiatrophy. Two of the infants had glaucoma; one also had a facial nevus flammeus and the other had cutis marmorata telangiectatica congenita of the face. A congenital pigmented nevus and a localized venous malformation constituted the remaining associated defects. This disease is an uncommon cutaneous vascular anomaly that is most often solitary but occasionally may be associated with other developmental defects.