Histopathological and epidemiological characteristics of patients with erythema exudativum multiforme major, Stevens—Johnson syndrome and toxic epidermal necrolysis

Histopathological and epidemiological characteristics of patients with erythema exudativum multiforme major, Stevens—Johnson syndrome and toxic epidermal necrolysis
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多形性渗出性红斑、Stevens-Johnson综合征和中毒性表皮坏死松解症患者的组织病理学和流行病学特征

DOI:
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发表时间:
1996
影响因子:
10.3
通讯作者:
E. Schöpf
E. Schöpf
中科院分区:
医学1区
文献类型:
--
作者:
Berthold Rzany;O. Hering;M. Mockenhaupt;Werner Schröder;E. Goerttler;J. Ring;E. Schöpf

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摘要 由于缺乏明确的标准,重度渗出性红斑 (EEMM)、史蒂文斯-约翰逊综合征 (SJS) 和中毒性表皮坏死松解症 (TEN) 的临床和组织病理学分类很困难。根据新的临床分类,来自 534 名 EEMM 患者的 219 份组织病理学标本中的 149 份 (68%)。 SJS 和 TEN 已经过审查。与临床表现以及任何既往感染史或药物摄入史进行比较。 1990年4月至1993年12月期间,所有患者均被纳入德国严重皮肤反应登记处。在临床诊断、性别和年龄方面,所检查的活组织检查和组织病理学标本总数之间没有发现差异。 149 个标本中有 28 个的切片无法诊断或太旧而无法正确评估。在九个病例中,提出了其他诊断。 111 张诊断为 EEMM(n=16)、SJS(n=34)和 TEN(n=61)的组织学切片被分类为表皮型多形红斑。在这 111 张载玻片中,可以发现坏死的角质形成细胞,从单个细胞到汇合的表皮坏死。表皮-真皮交界处显示出从空泡改变到表皮下水疱的变化。真皮浸润是浅表的并且大部分是血管周围的。它在 SJS 和 TEN 中稀疏,而在 EEMM 中更明显。在所有临床组中,偶尔会出现明显的乳头状真皮水肿。 111 例中有 59 例 (53%) 真皮中至少存在一种嗜酸性粒细胞。 111 个视野中有 11 个(10%)每个视野可见超过 10 个嗜酸性粒细胞。嗜酸性粒细胞在最严重的 TEN 患者中较少见,这些患者的皮肤表面积脱落超过 30%。在皮肤切片有或没有嗜酸性粒细胞的患者之间,没有发现药物摄入史或肺炎支原体、单纯疱疹和其他微生物感染史的差异。这项针对 EEMM 患者的皮肤病理学研究。 SJS 和 TEN 表明表皮型多形红斑与这些疾病的病理相关。
Summary The clinical and histopathological classification of erythema exudativum multiforme major (EEMM), Stevens‐Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are difficult, due to the lack of clear‐cut criteria. Based on a new clinical classification, 149 of 219 (68%) histopathological specimens, from a total of 534 patients with EEMM. SJS and TEN, have been reviewed. A comparison was made with the clinical picture, and any past history of infection or drug intake. All patients had been included in the German Registry of Severe Skin Reactions between April 1990 and December 1993. No differences could be found between the biopsies examined and the total number of histopathological specimens, concerning clinical diagnosis, gender and age. Sections from 28 of 149 specimens were not diagnostic or were too old to be properly evaluated. In nine cases, other diagnoses were proposed. One hundred and eleven of the histological slides with the diagnosis of EEMM (n= 16), SJS (n=34) and TEN (n=61), were classified as epidermal type of erythema multiforme. In these 111 slides, necrotic keratinocytes could be found, ranging from individual cells to confluent epidermal necrosis. The epidermo‐dermal junction showed changes ranging from vacuolar alteration up to subepidermal blisters. The dermal infiltrate was superficial and mostly perivascular. It was sparse in SJS and TEN, and more pronounced in EEMM. Oedema in the papillary dermis was evident occasionally in all clinical groups. In 59 of 111 cases (53%), at least one eosinophil was present in the dermis. In 11 of 111 (10%), more than 10 eosinophils per field could be seen. Eosinophils were less common in the patients with the most severe forms of TEN, in whom there was detachment of more than 30% of the skin surface area. No differences in the history for drug intake, or for infection with Mycoplasma pneumoniae, herpes simplex and other organisms, could be detected between patients with or without eosinophils in their skin sections. This dermatopathological study of patients with EEMM. SJS and TEN indicates that the epidermal type of erythema multiforme is the pathological correlate for these diseases.