Wong Anthony, Malvestiti Andrey Augusto, Hafner Mariana de Figueiredo Silva
Professor of Clinical Toxicology, Medical Director of Centro de Assistência Toxicológica (Ceatox), Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo (FMUSP), São Paulo, SP, Brazil.
Assistant Physician at the Ceatox, Hospital das Clínicas, FMUSP. Dermatologist at Hospital Israelita Albert Einstein (HIAE), São Paulo, SP, Brazil.
Rev Assoc Med Bras (1992). 2016 Sep-Oct;62(5):468-73. doi: 10.1590/1806-9282.62.05.468.
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are uncommon, acute and potentially life-threatening adverse cutaneous drug reactions. These pathologies are considered a hypersensitivity reaction and can be triggered by drugs, infections and malignancies. The drugs most often involved are allopurinol, some antibiotics, including sulfonamides, anticonvulsants such as carbamazepine, and some non-steroid anti-inflammatory drugs (NSAIDs). Necrosis of keratinocytes is manifested clinically by epidermal detachment, leading to scalded skin appearance. The rash begins on the trunk with subsequent generalization, usually sparing the palmoplantar areas. Macular lesions become purplish, and epidermal detachment occurs, resulting in flaccid blisters that converge and break, resulting in extensive sloughing of necrotic skin. Nikolsky's sign is positive in perilesional skin. SJS and TEN are considered to be two ends of the spectrum of one disease, differing only by their extent of skin detachment. Management of patients with SJS or TEN requires three measures: removal of the offending drug, particularly drugs known to be high-risk; supportive measures and active interventions. Early diagnosis of the disease, recognition of the causal agent and the immediate withdrawal of the drug are the most important actions, as the course of the disease is often rapid and fatal.
史蒂文斯-约翰逊综合征(SJS)和中毒性表皮坏死松解症(TEN)是罕见的、急性且可能危及生命的皮肤药物不良反应。这些病症被认为是一种超敏反应,可由药物、感染和恶性肿瘤引发。最常涉及的药物有别嘌醇、某些抗生素(包括磺胺类)、抗惊厥药如卡马西平以及一些非甾体抗炎药(NSAIDs)。角质形成细胞坏死在临床上表现为表皮剥脱,导致皮肤呈烫伤样外观。皮疹始于躯干,随后泛发,通常不累及掌跖部位。斑疹病变变为紫色,出现表皮剥脱,形成松弛性水疱,水疱融合破裂,导致大片坏死皮肤脱落。尼氏征在皮损周围皮肤呈阳性。SJS和TEN被认为是同一种疾病谱的两端,仅在皮肤剥脱程度上有所不同。SJS或TEN患者的治疗需要采取三项措施:停用致病药物,尤其是已知的高风险药物;支持性措施和积极干预。疾病的早期诊断、识别病因并立即停用药物是最重要的措施,因为疾病进程往往迅速且致命。