Wang Lin, Hong Kuo-Ching, Lin Fu-Chein, Yang Kuender D
Department of Pediatrics, Chang Gung Children's Hospital at Kaohsiung, Taiwan.
J Formos Med Assoc. 2003 Jan;102(1):55-8.
Erythema multiforme (EM) is an immune-mediated disease categorized into EM minor and EM major, also called Stevens-Johnson syndrome. The presence of mucosal involvement differentiates erythema multiforme major from erythema multiforme minor. Many drugs and agents can induce Stevens-Johnson syndrome. We report a case of Stevens-Johnson syndrome associated with Mycoplasma pneumoniae infection. Lymphopenia with a significant decrease of CD4+ T cells in the blood and predominant CD4+ T cells in the skin vesicular fluid was found. The improvement of lymphopenia was associated with disease recovery. In a retrospective chart review of patients treated in our hospital over the past 3 years, we found that 5 patients with Stevens-Johnson syndrome all had lymphopenia (< 1.50 x 10(9)/L; average 0.99 x 10(9)/L), whereas 13 other patients with erythema multiforme minor demonstrated normal lymphocyte counts (average 3.13 x 10(9)/L), with the exception of one patient with herpes infection showing lymphopenia. These results suggested that an immunopathogenesis involving redistribution of CD4+ T cells might contribute to the development of Stevens-Johnson syndrome. Further studies to investigate the involvement of CD4+ T cells in Stevens-Johnson syndrome may implicate a specific strategy to prevent fatal Stevens-Johnson syndrome.
多形红斑(EM)是一种免疫介导性疾病,分为轻症多形红斑和重症多形红斑,后者也称为史蒂文斯-约翰逊综合征。黏膜受累的情况可将重症多形红斑与轻症多形红斑区分开来。许多药物和制剂可诱发史蒂文斯-约翰逊综合征。我们报告1例与肺炎支原体感染相关的史蒂文斯-约翰逊综合征病例。发现患者血液中淋巴细胞减少,CD4+T细胞显著减少,而皮肤水疱液中以CD4+T细胞为主。淋巴细胞减少的改善与疾病恢复相关。在对我院过去3年治疗的患者进行的回顾性病历审查中,我们发现5例史蒂文斯-约翰逊综合征患者均有淋巴细胞减少(<1.50×10⁹/L;平均0.99×10⁹/L),而13例轻症多形红斑患者淋巴细胞计数正常(平均3.13×10⁹/L),只有1例疱疹感染患者出现淋巴细胞减少。这些结果提示,涉及CD4+T细胞重新分布的免疫发病机制可能促成了史蒂文斯-约翰逊综合征的发生。进一步研究CD4+T细胞在史蒂文斯-约翰逊综合征中的作用,可能会为预防致命性史蒂文斯-约翰逊综合征带来特定策略。