Background: Congenital leukemia cutis is rarely reported in the dermatology literature despite various authors citing 50% of infants with congenital leukemia have skin involvement. These seemingly disparate facts prompted a review of the world literature that was performed by searching the MEDLINE database from 1966 up to and through December 1992, reviewing Index Medicus for the years prior to computerized MEDLINE search, and reviewing appropriate case report references.
Observations: A 2-month premature male infant was born with 182 x 10(9)/L circulating monoblasts, prominent hepatosplenomegaly, and multiple firm blue and red cutaneous nodules.
Conclusion: Approximately 175 cases of congenital leukemia have been reported with at least 41 and possibly 56 cases identified with leukemia cutis. Specific cutaneous leukemic infiltrates occur in 25% to 30% of infants with congenital leukemia and usually appear as firm blue, red, or purple nodules in a generalized distribution. In contrast to the 50% incidence of gingival and oral infiltrates reported in adult monocytic leukemia, only 1% of congenital leukemia patients have oral involvement. Congenital leukemia cutis may precede other manifestations of leukemia by as much as 4 months. Whereas leukemia cutis is associated with a poor prognosis in adult leukemics, the natural history of congenital leukemia is not altered by leukemia cutis.