Giakas Alec, Holder Katherine, Galvan Bernardo, Stallworth James
Department of Pediatrics, University of South Carolina School of Medicine, Columbia, South Carolina, USA.
Department of Internal Medicine, Texas Tech University Health Sciences Center School of Medicine, Lubbock, Texas, USA.
BMJ Case Rep. 2023 Jan 12;16(1):e251989. doi: 10.1136/bcr-2022-251989.
Systemic lupus erythematosus (SLE) is an autoimmune disease with varying dermatological findings. We review a unique presentation of SLE with a literature review. A previously healthy early adolescent female presented with painful, oral mucosal bullae filled with sanguineous fluid. She endorsed a tender right knee, but examination revealed no additional abnormalities. CBC demonstrated severe pancytopaenia. Further workup, including Coombs positive RBCs and positive ANA, anti-Smith, and anti-dsDNA antibodies, confirmed Lupus as the aetiology of this patient's presentation. A form of blistering SLE and resultant thrombocytopaenia was likely responsible for the patient's oral manifestations. After receiving 60 g intravenous immunoglobulin and 3 days high-dose pulse corticosteroids, her dermatological symptoms resolved. Although cases of blistering SLE with mucosal bullae have been described in the literature, this is the first documented case of haemorrhagic mucosal bullae as the presenting symptom of thrombocytopaenia in SLE in a paediatric patient.
系统性红斑狼疮（SLE）是一种具有多种皮肤表现的自身免疫性疾病。我们通过文献回顾来审视SLE的一种独特表现形式。一名此前健康的青春期早期女性出现了充满血性液体的疼痛性口腔黏膜大疱。她自述右膝疼痛，但检查未发现其他异常。全血细胞计数显示严重全血细胞减少。进一步检查，包括库姆斯试验阳性的红细胞以及抗核抗体、抗史密斯抗体和抗双链DNA抗体阳性，确诊狼疮为本例患者临床表现的病因。一种水疱性SLE形式及由此导致的血小板减少可能是患者口腔表现的原因。在接受60克静脉注射免疫球蛋白和3天高剂量脉冲式皮质类固醇治疗后，她的皮肤症状得以缓解。尽管文献中已描述过伴有黏膜大疱的水疱性SLE病例，但这是首例记录在案的以出血性黏膜大疱作为小儿SLE血小板减少首发症状的病例。
