Dermatology Department, University Hospitals of Leicester NHS Trust, Leicester Royal Infirmary, Leicester, UK.
Histopathology Department, University Hospitals of Leicester NHS Trust, Leicester Royal Infirmary, Leicester, UK.
Clin Exp Dermatol. 2021 Oct;46(7):1299-1303. doi: 10.1111/ced.14648. Epub 2021 May 10.
Toxic epidermal necrosis (TEN)-like lupus is a rare condition characterized by epidermal loss and mucosal ulceration occurring in patients with acute severe flares of systemic lupus erythematosus. The clinical picture may mimic drug-induced Stevens-Johnson syndrome/TEN; however, the absence of a suitable culprit drug, and the context of acute lupus point to the correct diagnosis. In a case series of three patients, further discriminating features included a slower onset of epidermal loss, more limited mucosal ulceration and a lack of ocular involvement when compared with drug-induced TEN. Histology may show similar features, including basal layer vacuolation, apoptosis and full-thickness epidermal necrosis. Patients with TEN-like lupus may have additional features of lupus, and a lupus band on direct immunofluorescence. It is important to identify this condition correctly, so that these patients can be appropriately managed with early input from Rheumatologists and prompt treatment with high-dose combined immunosuppressant therapy.
中毒性表皮坏死松解症样狼疮是一种罕见的疾病，其特征是在系统性红斑狼疮急性严重发作时出现表皮脱落和黏膜溃疡。临床表现可能类似于药物诱导的史蒂文斯-约翰逊综合征/中毒性表皮坏死松解症；然而，缺乏合适的致病药物，以及急性狼疮的背景提示了正确的诊断。在三例患者的病例系列中，与药物诱导的中毒性表皮坏死松解症相比，进一步区分的特征包括表皮脱落的发病较慢、黏膜溃疡更有限且无眼部受累。组织学可能显示出类似的特征，包括基底细胞空泡化、细胞凋亡和全层表皮坏死。中毒性表皮坏死松解症样狼疮患者可能有狼疮的其他特征，以及直接免疫荧光检查的狼疮带。正确识别这种情况非常重要，以便风湿科医生能够及早介入并及时给予大剂量联合免疫抑制剂治疗。
