Wisuthsarewong Wanee, Soongswang Jarupim, Chantorn Rattanavalai
Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University Bangkoknoi, Bangkok, Thailand.
Pediatr Dermatol. 2011 Mar-Apr;28(2):115-21. doi: 10.1111/j.1525-1470.2011.01300.x. Epub 2011 Mar 1.
Neonatal lupus erythematosus is an uncommon maternal auto-antibody-associated disease characterized by cutaneous, cardiac, hepatic, hematological, neurological, and pulmonary involvement. A retrospective study was performed to review clinical manifestations, investigation results, outcomes of neonatal lupus erythematosus patients and their mothers at the Department of Pediatrics, Siriraj Hospital during 1993 to 2008. Seventeen neonatal lupus erythematosus patients (10 girls and seven boys) were identified. Cutaneous, cardiac, hepatobiliary, and hematological involvement was found in 70.6%, 64.7%, 52.9%, and 35.3% of infants, respectively. Skin lesions were erythematous patches (91.7%), subacute cutaneous lupus erythematosus (50%), petechiae (41.7%), persistent cutis marmorata (16.7%), and discoid lesions (8.3%). Congenital heart block was found in nine cases, and structural abnormalities were found in nine cases. All sera of patients were positive for antinuclear antibodies. Patients (87.5%) showed positive antiRo/SSA, and 50% had positive antiLa/SSB antibodies. Most neonatal lupus erythematosus mothers (64.7%) were asymptomatic. Five mothers were diagnosed with systemic lupus erythematosus, and one mother was diagnosed with mixed connective tissue disease. All maternal sera was positive for antinuclear antibodies and antiRo/SSA antibody. Seven patients required pacemaker implantation. The mortality rate was 11.8%, caused by congestive heart failure and pneumonia. Antinuclear antibody tests should be used as one of the screening tests in mothers or patients suspected of having neonatal lupus erythematosus.
新生儿红斑狼疮是一种罕见的与母体自身抗体相关的疾病，其特征为皮肤、心脏、肝脏、血液、神经和肺部受累。进行了一项回顾性研究，以回顾1993年至2008年期间诗里拉吉医院儿科新生儿红斑狼疮患者及其母亲的临床表现、检查结果和结局。共确定了17例新生儿红斑狼疮患者（10名女孩和7名男孩）。分别有70.6%、64.7%、52.9%和35.3%的婴儿出现皮肤、心脏、肝胆和血液系统受累。皮肤病变包括红斑斑块（91.7%）、亚急性皮肤型红斑狼疮（50%）、瘀点（41.7%）、持续性大理石样皮肤（16.7%）和盘状皮损（8.3%）。发现9例先天性心脏传导阻滞，9例有结构异常。所有患者血清抗核抗体均为阳性。患者抗Ro/SSA抗体阳性率为87.5%，抗La/SSB抗体阳性率为50%。大多数新生儿红斑狼疮母亲（64.7%）无症状。5名母亲被诊断为系统性红斑狼疮，1名母亲被诊断为混合性结缔组织病。所有母体血清抗核抗体和抗Ro/SSA抗体均为阳性。7例患者需要植入起搏器。死亡率为11.8%，由充血性心力衰竭和肺炎导致。抗核抗体检测应作为疑似患有新生儿红斑狼疮的母亲或患者的筛查试验之一。
