Assier H, Bastuji-Garin S, Revuz J, Roujeau J C
Department of Dermatology, Hôpital Henri-Mondor, Université, Paris XII, Créteil, France.
Arch Dermatol. 1995 May;131(5):539-43.
It was recently suggested that erythema multiforme (EM) majus and Stevens-Johnson syndrome (SJS) could be separated as two distinct clinical disorders with similar mucosal erosions but different patterns of cutaneous lesions. To test that hypothesis, we made a single-center retrospective study of severe EM with skin and mucosal involvement. Based on a review of clinical photographs, the skin lesions were reclassified as EM when these lesions were made of typical or raised atypical targets that were located on the extremities and/or the face, or as SJS when these lesions were made of flat atypical targets or purpuric maculae that were widespread or distributed on the trunk. Another investigator who was blinded for that clinical classification related each case to its more probable cause (eg, herpes infection or drug-induced reaction), by using scores derived from the medical charts.
The majority (80%) of 76 cases could be classified as one of the two disorders: 28 as EM (37%), 33 as SJS (43%), and 15 as "undetermined" (20%). By using causal scores, the 76 cases were classified as herpes-induced (n = 18 [24%]), drug-induced (n = 40 [52%]), and "other" (n = 18 [24%]). There was a strong correlation between the clinical classification and the probable cause (K = 0.87, P < .001). Specifically, EM was mostly related to herpes (17 of 28 cases) or to other causes (eight of 28 cases); however, EM was rarely related to drugs (three of 28 cases), while SJS was nearly always related to drugs (28 of 33 cases) and never to herpes.
The results of this study support the suggestion that EM with mucosal lesions and SJS could be distinguished on the basis of two different clinical patterns. In addition, a strong relationship was observed between each pattern and specific causes. This is one more piece of evidence that suggests that EM with mucosal lesions and SJS are actually two different diseases.
最近有人提出，重症多形红斑（EM）和史蒂文斯-约翰逊综合征（SJS）可被区分为两种不同的临床疾病，它们有相似的黏膜糜烂，但皮肤损害模式不同。为验证这一假说，我们对累及皮肤和黏膜的重症EM进行了一项单中心回顾性研究。通过回顾临床照片，当皮肤损害由位于四肢和/或面部的典型或隆起的非典型靶形损害构成时，将其重新分类为EM；当这些损害由广泛分布于躯干或遍布全身的扁平非典型靶形损害或紫癜性斑构成时，则分类为SJS。另一位对该临床分类不知情的研究者，通过使用从病历中得出的评分，将每个病例与其最可能的病因（如疱疹感染或药物性反应）相关联。
76例病例中的大多数（80%）可被分类为这两种疾病之一：28例为EM（37%），33例为SJS（43%），15例为“未确定”（20%）。根据病因评分，76例病例被分类为疱疹诱发（n = 18 [24%]）、药物诱发（n = 40 [52%]）和“其他”（n = 18 [24%]）。临床分类与可能的病因之间存在很强的相关性（K = 0.87，P <.001）。具体而言，EM大多与疱疹（28例中的17例）或其他病因（28例中的8例）相关；然而，EM很少与药物相关（28例中的3例），而SJS几乎总是与药物相关（33例中的28例），且从未与疱疹相关。
本研究结果支持以下观点，即伴有黏膜损害的EM和SJS可基于两种不同的临床模式进行区分。此外，观察到每种模式与特定病因之间存在密切关系。这进一步证明伴有黏膜损害的EM和SJS实际上是两种不同的疾病。
