Scheker Luis Emilio, Kasteler J Scott, Callen Jeffrey P
Division of Dermatology, Department of Medicine, University of Louisville School of Medicine, Kentucky 40202, USA.
Pediatr Dermatol. 2003 Mar-Apr;20(2):164-6. doi: 10.1046/j.1525-1470.2003.20216.x.
Neonatal lupus erythematosus (NLE) is an autoimmune disease characterized primarily by transient skin lesions and/or permanent congenital heart block. Other clinical findings include self-limited cytopenias and liver disease. The syndrome results from the passive transfer of maternal anti-SSA, anti-SSB, or anti-U1RNP autoantibodies to the fetus across the placenta. The cutaneous manifestations are generally analogous to those of subacute cutaneous lupus erythematosus (SCLE) and consist of small, erythematous macules that progress to annular plaques with delicate scaling. The skin lesions usually resolve within the first 6 months of life as maternal autoantibodies are cleared from the infant's circulation. We describe a patient with cutaneous NLE with hepatic and hematologic manifestations. The clinical presentation was atypical, with splenomegaly and petechiae at birth followed by a crusting, papulosquamous skin eruption of the scalp and face mimicking Langerhans cell histiocytosis (LCH).
新生儿红斑狼疮（NLE）是一种自身免疫性疾病，主要特征为短暂性皮肤病变和/或永久性先天性心脏传导阻滞。其他临床表现包括自限性血细胞减少和肝病。该综合征是由于母体抗SSA、抗SSB或抗U1RNP自身抗体通过胎盘被动转移至胎儿所致。皮肤表现通常类似于亚急性皮肤型红斑狼疮（SCLE），由小的红斑性斑疹组成，逐渐发展为有精细鳞屑的环状斑块。随着母体自身抗体从婴儿循环中清除，皮肤病变通常在出生后的头6个月内消退。我们描述了一名患有皮肤型NLE并伴有肝脏和血液学表现的患者。临床表现不典型，出生时伴有脾肿大和瘀点，随后头皮和面部出现结痂性丘疹鳞屑性皮疹，类似朗格汉斯细胞组织细胞增多症（LCH）。
