Kardaun Sylvia H, Kuiper Hilde, Fidler Vaclav, Jonkman Marcel F
Department of Dermatology, Reference Center for Cutaneous Adverse Drug Reactions, University Medical Center Groningen, University of Groningen, Hanzeplein 1, Groningen, The Netherlands.
J Cutan Pathol. 2010 Dec;37(12):1220-9. doi: 10.1111/j.1600-0560.2010.01612.x. Epub 2010 Aug 25.
Acute generalized exanthematous pustulosis (AGEP) represents a severe, acute, pustular skin reaction that is most often induced by drugs. AGEP can be difficult to differentiate from generalized pustular psoriasis (GPP) both clinically and histopathologically. We present a systematic description of the histopathological spectrum of AGEP and GPP with a focus on discriminating features.
A retrospective, descriptive, comparative histopathological study was completed utilizing step sections of 43 biopsies of 29 cases with a validated diagnosis of probable or definite AGEP and 24 biopsies of 19 cases with an established diagnosis of GPP.
In AGEP, biopsies from erythema and pustules showed minor differences, whereas histopathology of the acute stage of GPP showed major differences compared to the chronic stage. Comparing AGEP and GPP, the presence of eosinophils, necrotic keratinocytes, a mixed interstitial and mid-dermal perivascular infiltrate and absence of tortuous or dilated blood vessels were in favor of AGEP. Moreover, chronic GPP was characterized by prominent epidermal psoriatic changes. The frequency of a psoriatic background of AGEP patients in our study was higher than that of psoriasis in the general population. However, histopathology of a subgroup of AGEP patients with a personal history of psoriasis revealed no significant differences from the other AGEP patients.
The spectrum of histopathological features of both AGEP and GPP is presented. Despite considerable overlap, subtle consistent histopathological differences and the grade of severity of specific features can help in differentiation. We could neither substantiate earlier reports that follicular pustules exclude AGEP nor did we see vasculitis as a specific feature in AGEP. Our study also supports the concept that AGEP is a separate entity that is distinct from GPP.
急性泛发性脓疱性皮病（AGEP）是一种严重的急性脓疱性皮肤反应，最常由药物引起。AGEP在临床和组织病理学上都可能难以与泛发性脓疱型银屑病（GPP）相鉴别。我们对AGEP和GPP的组织病理学谱进行了系统描述，重点关注鉴别特征。
利用29例经证实诊断为可能或确诊AGEP的病例的43份活检标本的连续切片以及19例已确诊GPP的病例的24份活检标本，完成了一项回顾性、描述性、对比性组织病理学研究。
在AGEP中，红斑和脓疱部位的活检标本显示差异较小，而GPP急性期的组织病理学与慢性期相比差异较大。比较AGEP和GPP，嗜酸性粒细胞、坏死角质形成细胞、混合性间质和真皮中部血管周围浸润的存在以及无迂曲或扩张血管有利于AGEP的诊断。此外，慢性GPP的特征是表皮有明显的银屑病样改变。在我们的研究中，AGEP患者有银屑病背景的频率高于一般人群中的银屑病发病率。然而，有银屑病个人史的AGEP患者亚组的组织病理学与其他AGEP患者无显著差异。
呈现了AGEP和GPP的组织病理学特征谱。尽管存在相当大的重叠，但细微一致的组织病理学差异以及特定特征的严重程度等级有助于鉴别。我们既无法证实早期关于毛囊脓疱可排除AGEP的报道，也未在AGEP中发现血管炎作为一个特定特征。我们的研究也支持AGEP是一个与GPP不同的独立实体的概念。
