Courville P, Kupfer I, Gilbert D, Thomine E, Metayer J, Joly P
Service d'Anatomie Pathologique, Unité INSERM U519, CHU Charles Nicolle, Rouen.
Ann Pathol. 2000 Dec;20(6):564-9.
The aim of this study was to evaluate the histological findings observed in patients with bullous pemphigoid in whom the diagnosis of bullous pemphigoid could be confirmed by direct immunofluorescence and immunoblot serum analysis. Seven histological criteria were considered for selection of skin biopsy specimens: 1) cleavage of dermal epidermal junction; 2) migration of eosinophils along dermal epidermal junction; 3) presence of intra epidermal eosinophils (with or without associated spongiosis); 4) absence of keratinocyte necrosis; 5) absence of acantholysis; 6) absence of dermal fibrosis; 7) absence of vasculitis. Depending on the number of criteria observed the histological picture was considered as: highly suggestive, suggestive or poorly suggestive of bullous pemphigoid. The histological picture was considered as highly suggestive in 50% of cases, suggestive or poorly suggestive in 37% and 13% of cases respectively. Migration of eosinophils along dermal epidermal junction was observed in 23 biopsy specimens (50%). Histological findings considered as poorly suggestive of bullous pemphigoid consisted of a prurigo-like or an eczematous-like or a drug induced-like picture or no specific cutaneous erosions. An histological picture highly suggestive of bullous pemphigoid was observed in 67% of patients whose serum contained anti-BPAG2 antibodies and in only 36% patients of without anti-BPAG2 antibodies (p=0,04). On the contrary, only one bullous pemphigoid patient (4%) with circulating anti-BPAG2 antibodies had a histological picture poorly suggestive of bullous pemphigoid. These findings are in accordance with the pathogenic properties of anti-BPAG2 antibodies demonstrated in animal models. This study showed that: 1) typical histological findings of bullous pemphigoid are only observed in 50% of skin biopsy specimens. 2) The diagnosis of bullous pemphigoid should be considered in elderly patients even when a poorly specific prurigo-like or eczematous-like histological picture is observed. Moreover, it underlines the usefulness of direct immunofluorescence of skin biopsy specimens and indirect immunofluorescence and immunoblot analysis of serum in such atypical cases of bullous pemphigoid.
本研究的目的是评估在大疱性类天疱疮患者中观察到的组织学发现，这些患者的大疱性类天疱疮诊断可通过直接免疫荧光和免疫印迹血清分析得以证实。选择皮肤活检标本时考虑了七个组织学标准：1）真皮表皮交界处的分裂；2）嗜酸性粒细胞沿真皮表皮交界处迁移；3）表皮内嗜酸性粒细胞的存在（伴有或不伴有相关的海绵形成）；4）角质形成细胞坏死的缺失；5）棘层松解的缺失；6）真皮纤维化的缺失；7）血管炎的缺失。根据观察到的标准数量，组织学表现被认为对大疱性类天疱疮具有高度提示性、提示性或低提示性。组织学表现在50%的病例中被认为具有高度提示性，在37%和13%的病例中分别为提示性或低提示性。在23个活检标本（50%）中观察到嗜酸性粒细胞沿真皮表皮交界处迁移。被认为对大疱性类天疱疮低提示性的组织学发现包括类痒疹样、类湿疹样或药物诱导样表现或无特异性皮肤糜烂。在血清含有抗BPAG2抗体的患者中，67%观察到高度提示大疱性类天疱疮的组织学表现，而在无抗BPAG2抗体的患者中仅36%观察到（p = 0.04）。相反，仅有一名循环抗BPAG2抗体的大疱性类天疱疮患者（4%）具有对大疱性类天疱疮低提示性的组织学表现。这些发现与动物模型中证实的抗BPAG2抗体的致病特性一致。本研究表明：1）大疱性类天疱疮的典型组织学发现仅在50%的皮肤活检标本中观察到。2）即使观察到特异性低的类痒疹样或类湿疹样组织学表现，老年患者也应考虑大疱性类天疱疮的诊断。此外，它强调了在这种非典型大疱性类天疱疮病例中皮肤活检标本的直接免疫荧光以及血清的间接免疫荧光和免疫印迹分析的有用性。
