Gardner Kerry M, Crawford Richard I
1 Departments of Dermatology and Skin Science, University of British Columbia, Vancouver, BC, Canada.
2 Departments of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, BC, Canada.
J Cutan Med Surg. 2018 Jan/Feb;22(1):22-24. doi: 10.1177/1203475417722734. Epub 2017 Jul 18.
It has been postulated that periodic acid-Schiff staining of basement membrane can predict direct immunofluorescence patterns seen in epidermolysis bullosa acquisita and bullous pemphigoid. It has also been suggested that the type of inflammatory infiltrate or presence of fraying of basal keratinocytes may differentiate these two conditions.
In this study, we aimed to confirm these observations.
We reviewed 13 cases of direct immunofluorescence-confirmed epidermolysis bullosa acquisita and 19 cases of direct immunofluorescence-confirmed bullous pemphigoid, all with a subepidermal blister in the routinely processed specimen. The gold standard for diagnosis of epidermolysis bullosa acquisita vs bullous pemphigoid was taken to be identification of immune deposits on the dermal side ('floor' for epidermolysis bullosa acquisita) or the epidermal side ('roof' for bullous pemphigoid) of the salt-split direct immunofluorescence specimen. Our tests to distinguish epidermolysis bullosa acquisita from bullous pemphigoid on the routinely processed biopsy included periodic acid-Schiff basement membrane on the blister roof, neutrophilic infiltrate, lack of eosinophilic infiltrate, and absence of keratinocyte fraying.
Sensitivity and specificity for each test were as follows: periodic acid-Schiff staining of roof (sensitivity 25%, specificity 95%), neutrophilic infiltrate (sensitivity 54%, specificity 74%), lack of eosinophilic infiltrate (sensitivity 92%, specificity 68%), and absence of keratinocyte fraying (sensitivity 62%, specificity 58%).
Features in the routinely processed biopsy were unable to reliably distinguish between epidermolysis bullosa acquisita and bullous pemphigoid. Direct immunofluorescence on salt-split skin remains the standard for differentiation.
据推测，基底膜的过碘酸-希夫染色可预测获得性大疱性表皮松解症和大疱性类天疱疮中所见的直接免疫荧光模式。也有人提出，炎症浸润的类型或基底角质形成细胞的磨损情况可能有助于区分这两种疾病。
在本研究中，我们旨在证实这些观察结果。
我们回顾了13例经直接免疫荧光确诊的获得性大疱性表皮松解症病例和19例经直接免疫荧光确诊的大疱性类天疱疮病例，所有病例在常规处理的标本中均有表皮下水疱。获得性大疱性表皮松解症与大疱性类天疱疮诊断的金标准是在盐裂直接免疫荧光标本的真皮侧（获得性大疱性表皮松解症的“底部”）或表皮侧（大疱性类天疱疮的“顶部”）识别免疫沉积物。我们在常规处理的活检标本上区分获得性大疱性表皮松解症和大疱性类天疱疮的检测方法包括水疱顶部的过碘酸-希夫基底膜染色、中性粒细胞浸润、无嗜酸性粒细胞浸润以及角质形成细胞无磨损。
每项检测的敏感性和特异性如下：顶部过碘酸-希夫染色（敏感性25%，特异性95%）、中性粒细胞浸润（敏感性54%，特异性74%）、无嗜酸性粒细胞浸润（敏感性92%，特异性68%）以及角质形成细胞无磨损（敏感性62%，特异性58%）。
常规处理的活检标本中的特征无法可靠地区分获得性大疱性表皮松解症和大疱性类天疱疮。盐裂皮肤的直接免疫荧光检查仍然是鉴别诊断的标准。
