Yen A, Sanchez R, Oblender M, Raimer S
Department of Dermatology, University of Texas Medical Branch, Galveston, USA.
J Am Acad Dermatol. 1996 Feb;34(2 Pt 2):375-8. doi: 10.1016/s0190-9622(07)80012-0.
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.
婴儿白血病仅占儿童白血病的3%。皮肤白血病发生于25%至30%的先天性白血病婴儿中，与急性髓系白血病的关联比急性淋巴细胞白血病更为常见。我们描述了一名婴儿，其在2周大时出现色素沉着斑，在4周大时这些斑显示出 Darier 征。该患者在10周大时被诊断为急性淋巴细胞白血病，早期前B细胞型。在那个年龄检查发现，头皮、面部和四肢有1至2厘米、质地坚硬、轻度压痛的结节聚集，躯干受累较轻，面部和眼睑明显硬结。从躯干浸润较轻的病变处引出了 Darier 征。组织学检查显示由多形性、未分化细胞组成的密集单形性浸润。吉姆萨染色未发现肥大细胞。据我们所知，该病例是首例报告的显示 Darier 征的皮肤白血病。
