Nijhawan A, Baselga E, Gonzalez-Ensenat M A, Vicente A, Southern J F, Camitta B M, Esterly N B, Drolet B A
Department of Dermatology, Medical College of Wisconsin, 9200 W Wisconsin Ave, Milwaukee, WI 53226, USA.
Arch Dermatol. 2001 Jun;137(6):760-3.
Infants with Down syndrome are at increased risk for hematologic abnormalities, including leukemoid reaction, transient myeloproliferative disorder, and congenital leukemia. The differential diagnosis of a vesiculopustular eruption in an infant with Down syndrome and these hematologic abnormalities is broad and includes benign, self-limited disorders as well as life-threatening infections.
We describe 3 newborns with Down syndrome and vesiculopustular eruptions associated with myeloproliferative disorders during the neonatal period. These lesions differ from other neonatal vesicular eruptions in that they have a unique distribution, display pathergy, and contain immature hematopoietic cells similar to circulating blast cells. Resolution occurs without treatment as the hematologic disorder subsides.
Infants with Down syndrome and hematologic abnormalities may have a cutaneous eruption that has characteristic clinical and histopathologic findings. It is possible that this eruption has been unrecognized in the past because of its self-limited course. Whether this eruption is a prognostic factor for the subsequent development of leukemia is uncertain.
唐氏综合征婴儿发生血液学异常的风险增加,包括类白血病反应、短暂性骨髓增殖性疾病和先天性白血病。患有唐氏综合征且有这些血液学异常的婴儿出现水疱脓疱疹的鉴别诊断范围广泛,包括良性自限性疾病以及危及生命的感染。
我们描述了3例患有唐氏综合征的新生儿,他们在新生儿期出现了与骨髓增殖性疾病相关的水疱脓疱疹。这些皮疹与其他新生儿水疱疹不同,其分布独特,显示同形反应,并且含有类似于循环原始细胞的未成熟造血细胞。随着血液学疾病消退,皮疹未经治疗即可消退。
患有唐氏综合征和血液学异常的婴儿可能会出现具有特征性临床和组织病理学表现的皮肤疹。过去可能由于其自限性病程而未被认识到这种皮疹。这种皮疹是否是随后发生白血病的预后因素尚不确定。