From the Division of Dermatology, Department of Internal Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
J Clin Rheumatol. 2019 Aug;25(5):224-231. doi: 10.1097/RHU.0000000000000830.
BACKGROUND/OBJECTIVE: Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN)-like lupus erythematosus is a hyperacute and life-threatening form of cutaneous lupus erythematosus. Because of its rarity, little is known about this entity. We aimed to evaluate the clinical characteristics, laboratory findings, systemic manifestations, treatments, and outcome of SJS/ TEN-like lupus erythematosus.
We conducted a chart review study from July 2002 to September 2016 of all patients diagnosed with SJS/TEN-like lupus erythematosus who presented with gradual epidermal necrolysis without clear drug or infectious culprit. We evaluate for clinical features, extracutaneous involvement, Systemic Lupus Erythematosus Disease Activity Index, histologic findings, immunofluorescence pattern, serologic abnormalities, treatment, outcome, and recurrence of SJS/TEN-like lupus erythematosus.
Of 9074 patients diagnosed with cutaneous lupus erythematosus and/or systemic lupus erythematosus, 6 patients justified the diagnosis of SJS/TEN-like lupus erythematosus (5 SJS/TEN-like acute cutaneous lupus erythematosus, 1 TEN-like subacute cutaneous lupus erythematosus), accounting for 0.07%. Fifty percent had epidermal necrolysis as the initial presentation of lupus with a median time from onset of 1.5 months (0-48 months). The median duration between initial rash and epidermal detachment was 4.5 days (3-14 days). All had internal organ involvement (hematologic and renal) and high Systemic Lupus Erythematosus Disease Activity Index score (median, 19.5 [16-24]). Most recovered with systemic corticosteroids, antimalarial drugs, and/or immunosuppressants. None had disease recurrence.
This is the largest single series of patients with SJS/TEN-like lupus erythematosus. Skin damage is an indicator of disease activity, and careful search for extracutaneous involvement to prevent further complications is mandatory.
背景/目的：史蒂文斯-约翰逊综合征（SJS）/中毒性表皮坏死松解症（TEN）样狼疮是一种超急性且危及生命的皮肤狼疮。由于其罕见性，对该疾病知之甚少。我们旨在评估 SJS/TEN 样狼疮的临床特征、实验室检查结果、全身表现、治疗方法和结局。
我们进行了一项回顾性病例研究，纳入了 2002 年 7 月至 2016 年 9 月期间所有因逐渐出现表皮坏死而无明确药物或感染原因的 SJS/TEN 样狼疮患者。我们评估了患者的临床特征、皮肤外表现、系统性红斑狼疮疾病活动指数、组织学表现、免疫荧光模式、血清学异常、治疗方法、结局以及 SJS/TEN 样狼疮的复发情况。
在 9074 例诊断为皮肤狼疮和/或系统性红斑狼疮的患者中，有 6 例符合 SJS/TEN 样狼疮的诊断（5 例 SJS/TEN 样急性皮肤狼疮，1 例 TEN 样亚急性皮肤狼疮），占比为 0.07%。有 50%的患者以皮肤坏死为狼疮的首发表现，发病中位时间为 1.5 个月（0-48 个月）。首发皮疹至表皮脱落的中位时间为 4.5 天（3-14 天）。所有患者均有内脏器官受累（血液和肾脏）和高系统性红斑狼疮疾病活动指数评分（中位数，19.5[16-24]）。大多数患者接受全身性皮质类固醇、抗疟药物和/或免疫抑制剂治疗后康复。无一例患者出现疾病复发。
这是 SJS/TEN 样狼疮患者最大的单一系列病例。皮肤损害是疾病活动的指标，必须仔细寻找皮肤外表现以预防进一步的并发症。
