Nar Iraz, Surmeli-Onay Ozge, Aytac Selin, Talim Beril, Kiper Pelin Ozlem, Boduroglu Koray, Yurdakok Murat
Department of Pediatrics, Faculty of Medicine, Hacettepe University, Ankara, Turkey.
Indian J Pediatr. 2014 Apr;81(4):391-3. doi: 10.1007/s12098-013-1192-7. Epub 2013 Aug 16.
Transient myeloproliferative disorder (TMD) typically presents with pancytopenia, hepatosplenomegaly, and immature circulating white blood cells, and affects approximately 10 % of neonates with Down syndrome. The authors report a neonate with Down syndrome who developed acute widespread pustular eruptions as a sign of TMD. The white blood cell counts on the first day of life were markedly elevated, with blasts seen on examination of the peripheral blood smear. And the patient was noted to have a few erythematous papules and pustules especially on the face. On the following days pathergy positive crusted papules and pustules were increased and spread to trunk and extremities. Skin biopsy specimens showed pustular dermatitis, with subcorneal vesiculopustules and perivascular inflammation in superficial dermis. These lesions improved parallel with the hematologic improvement within two weeks. The authors aim to alert clinicians about this uncommon cause of vesiculopustular eruption with the present illustrative case and review the literature.
短暂性骨髓增殖性疾病（TMD）通常表现为全血细胞减少、肝脾肿大以及循环中出现未成熟白细胞，约10%的唐氏综合征新生儿会受其影响。作者报告了一名患有唐氏综合征的新生儿，其出现急性广泛脓疱疹作为TMD的体征。出生第一天白细胞计数显著升高，外周血涂片检查可见原始细胞。且该患者尤其面部有一些红斑丘疹和脓疱。在接下来的几天里，同形反应阳性的结痂丘疹和脓疱增多并蔓延至躯干和四肢。皮肤活检标本显示脓疱性皮炎，角质层下有囊泡脓疱以及真皮浅层血管周围炎症。这些皮损在两周内随着血液学指标的改善而好转。作者旨在通过本病例说明提醒临床医生注意这种导致囊泡脓疱疹的不常见病因，并对文献进行综述。
