Brazzelli Valeria, Segal Aviad, Bernacca Carlotta, Tchich Adi, Bolcato Vittorio, Croci Giorgio, Mina Tommaso, Zecca Marco, Zanette Simona, Stronati Mauro
Institute of Dermatology, IRCCS Policlinico San Matteo Foundation, University of Pavia, Pavia, Italy.
Unit of Anatomic Pathology, IRCCS Policlinico San Matteo Foundation, University of Pavia, Pavia, Italy.
Pediatr Dermatol. 2019 Sep;36(5):702-706. doi: 10.1111/pde.13931. Epub 2019 Jul 29.
Transient myeloproliferative disorder (TMD) is a spontaneously resolving clonal myeloid proliferation characterized by circulating megakaryoblasts in the peripheral blood that is restricted to neonates with Down syndrome (DS) or those with trisomy 21 mosaicism. Cutaneous manifestations of TMD are observed in only 5% of affected neonates and present as a diffuse eruption of erythematous, crusted papules, papulovesicles, and pustules, often with prominent and initial facial involvement. We describe the case of a male infant with DS and TMD, associated with a vesiculopustular eruption, which appeared on day 36 of life, and review previous cases.
短暂性骨髓增殖性疾病(TMD)是一种可自发缓解的克隆性髓系增殖性疾病,其特征是外周血中出现循环巨核母细胞,仅见于唐氏综合征(DS)新生儿或21三体嵌合体新生儿。仅5%的受影响新生儿会出现TMD的皮肤表现,表现为弥漫性红斑、结痂丘疹、丘疱疹和脓疱疹,通常最初面部受累明显。我们描述了一例患有DS和TMD的男婴病例,该病例伴有水疱脓疱疹,于出生后第36天出现,并回顾了既往病例。