Aleukemic Congenital Leukemia Cutis

Abstract: A newborn girl had typical “blueberry muffin” skin lesions,which showed histopathologic features of myelomonocytic leukemia cutis.We could not demonstrate leukemic infiltration of bone marrow in four aspi-rates. Her course was complicated with primary pulmonary hypertension,which led to death at 7 months of age. We emphasize the persistence of skinlesions in the absence of bone marrow infiltration by leukemia throughout the course of the disease. The term congenital leukemia has been applied toinstances of leukemia that develop within the first 4weeks of life (1). This diagnosis implies the proliferationof immature white blood cells in the absence of anyother disease that can cause leukemoid proliferationsmimicking congenital leukemia. Such proliferationsmay be seen in fetomaternal blood incompatibility andcongenital infections such as syphilis, toxoplasmosis,rubella, or infections caused by cytomegalovirus, herpessimplex, or bacteria (1). Furthermore, patients with Downsyndrome or chromosome 21 anomalies may show atypical transient myeloproliferative disorder (TMD) (1).Infiltration of the skin has been documented in 25–30% of patients with congenital leukemia (2). Leukemiacutis may be the first manifestation of congenital leukemia,and may even precede blood or bone marrow leukemiaby several weeks or months (3). Most cases of congenitalleukemia are of the myelogenous type. The natural his-tory of congenital leukemia is usually fatal, although afew patients have experienced temporary or permanentspontaneous remissions (4,5). We report a newborn girlwho had skin lesions consistent with leukemia cutis, butwithout blood or bone marrow leukemia until 7 monthsof age, when she died of unrelated cardiac disease.