Buster Kesha J, Burford Holly N, Stewart Faith A, Sellheyer Klaus, Hughey Lauren C
Department of Dermatology, University of Alabama at Birmingham, 1530 3rd Ave S, EFH Set 414, Birmingham, AL 35294-0009, USA.
Cutis. 2013 Aug;92(2):83-7.
Sclerema neonatorum (SN) is a rare neonatal panniculitis that typically develops in severely ill, preterm newborns within the first week of life and often is fatal. It usually occurs in preterm newborns with delivery complications such as respiratory distress or maternal complications such as eclampsia. Few clinical trials have been performed to address potential treatments. Successful treatment has been achieved via exchange transfusion (ET), but its use in neonates is declining. Similar to ET, intravenous immunoglobulin (IVIG) enhances both humoral and cellular immunity and thus may decrease mortality associated with SN. We report a case of SN in a term newborn who subsequently developed septicemia. Biopsy showed subcutaneous, needle-shaped clefts without associated necrosis, inflammation, or calcifications. Treatment with IVIG led to notable but short-term clinical improvement. Sclerema neonatorum remains a poorly understood and difficult to treat neonatal disorder. Although IVIG did not prevent our patient's death, further studies are needed to determine its clinical utility in the treatment of this rare disorder.
新生儿硬肿症(SN)是一种罕见的新生儿皮下脂肪炎,通常发生在病情严重的早产新生儿出生后的第一周内,且往往是致命的。它通常发生在有分娩并发症(如呼吸窘迫)的早产新生儿或有母体并发症(如子痫)的早产新生儿中。很少有临床试验针对潜在治疗方法进行研究。通过换血疗法(ET)已取得成功治疗,但它在新生儿中的使用正在减少。与ET类似,静脉注射免疫球蛋白(IVIG)可增强体液免疫和细胞免疫,因此可能降低与SN相关的死亡率。我们报告了一例足月儿患SN且随后发生败血症的病例。活检显示皮下有针状裂隙,无相关坏死、炎症或钙化。IVIG治疗导致了显著但短期的临床改善。新生儿硬肿症仍然是一种了解不足且难以治疗的新生儿疾病。尽管IVIG未能防止我们患者的死亡,但仍需要进一步研究以确定其在治疗这种罕见疾病中的临床效用。