Infantile leukemia accounts for only 3% of childhood leukemia. Leukemia cutis occurs in 25% to 30% of infants with congenital leukemia and is more frequently associated with acute myeloid leukemia than with acute lymphoblastic leukemia. We describe an infant in whom hyperpigmented macules that developed when the patient was 2 weeks old demonstrated Darier's sign when he was 4 weeks old. Acute lymphoblastic leukemia, early pre-B-cell type, was diagnosed when the patient was 10 weeks old. Examination at that age revealed 1 to 2 cm, firm, mildly tender nodules clustered on the scalp, face, and extremities, less severe involvement of the trunk, and marked induration of the face and eyelids. Darier's sign was elicited from the less infiltrated truncal lesions. Histologic examination revealed a dense monomorphous infiltrate consisting of pleomorphic, undifferentiated cells. No mast cells were revealed by Giemsa staining. This case is to our knowledge the first reported example of leukemia cutis demonstrating Darier's sign.
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