Department of Dermatology, Venereology and Allergology, University Hospital Leipzig, Philipp-Rosenthal-Str. 23-25, 04103 Leipzig, Germany.
Br J Dermatol. 2012 Mar;166(3):575-600. doi: 10.1111/j.1365-2133.2011.10705.x.
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe cutaneous adverse reactions with high morbidity and mortality. Some expressions of lupus erythematosus (LE) may cause enormous difficulties in differentiating them from SJS and TEN by showing large areas of sheet-like epidermal necrosis.
To evaluate clinically and histopathologically probable or definite cases of SJS/TEN with a history of systemic or other LE [(S)LE].
This was a retrospective analysis of validated cases of SJS/TEN with a history of (S)LE, based on a large population-based national registry.
Among 1366 patients with SJS/TEN, 17 with a sufficiently documented history of (S)LE and representative histological material could be identified, suggesting a considerable over-representation of LE in patients with SJS/TEN. Eight of these showed clinically and/or histopathologically some LE-characteristic features interfering with the diagnosis of SJS/TEN. Differentiation could be elaborated on clinical and histopathological grounds: four patients were classified as SJS/TEN with a preceding (S)LE exacerbation and/or LE-typical histopathological features, and four as 'TEN-like' (S)LE.
Most patients with SJS/TEN and a history of (S)LE demonstrate clinical and histopathological properties allowing clear differentiation. However, occasionally acute cutaneous manifestations of (S)LE and SJS/TEN can be phenotypically similar, caused by extensive epidermal necrosis. Although no feature by itself is conclusive, a combination of recent (S)LE exacerbation, evident photodistribution, annular lesions and absent or only mild focal erosive mucosal involvement may favour LE over SJS/TEN clinically. Histopathologically, in particular, junctional vacuolar alteration, and the presence of solitary necrotic keratinocytes at lower epidermal levels, combined with moderate to dense periadnexal and perivascular lymphocytic infiltrates with a variable presence of melanophages, and mucin point to a LE-related origin.
史蒂文斯-约翰逊综合征（SJS）和中毒性表皮坏死松解症（TEN）是具有高发病率和死亡率的严重皮肤不良反应。一些红斑狼疮（LE）的表现可能导致巨大的困难，从 SJS 和 TEN 区分它们通过表现大片状表皮坏死。
评估临床上和组织病理学上可能或明确的 SJS/TEN 病例，有系统性或其他 LE 的病史[（S）LE]。
这是基于大型基于人群的国家登记处的回顾性分析，有（S）LE 病史的 SJS/TEN 验证病例。
在 1366 例 SJS/TEN 患者中，有 17 例有充分记录的（S）LE 病史和代表性组织学材料，可以确定，提示 SJS/TEN 患者中 LE 的代表性过高。其中 8 例表现出一些临床上和/或组织病理学上的 LE 特征性特征，干扰了 SJS/TEN 的诊断。可以根据临床和组织病理学依据进行区分：4 例患者被归类为 SJS/TEN，有先前的（S）LE 加重和/或 LE 典型的组织病理学特征，4 例作为“TEN 样”（S）LE。
大多数 SJS/TEN 患者有病史（S）LE 表现出明确区分的临床和组织病理学特征。然而，偶尔急性皮肤表现（S）LE 和 SJS/TEN 可能在表型上相似，由广泛的表皮坏死引起。尽管没有一个特征本身是决定性的，但近期（S）LE 加重、明显的光分布、环状病变和不存在或仅有轻微局灶性侵蚀性黏膜受累的组合可能在临床上更有利于 LE 而不是 SJS/TEN。组织病理学上，特别是，连接空泡改变，以及较低表皮水平存在单个坏死角质形成细胞，结合中度至密集的附属器和血管周围淋巴细胞浸润，伴有不同程度的黑素细胞和粘蛋白，提示与 LE 相关的起源。
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