Mandel Victor Desmond, Farnetani Francesca, Vaschieri Cristina, Manfredini Marco, Cesinaro Anna Maria, Giusti Francesca, Pellacani Giovanni
Department of Dermatology, University of Modena and Reggio Emilia, Modena, Italy.
Department of Anatomic Pathology, Azienda Ospedaliero-Universitaria Policlinico, Modena, Italy.
J Dermatol. 2016 Aug;43(8):940-3. doi: 10.1111/1346-8138.13314. Epub 2016 Mar 6.
We report the case of a 74-year-old man affected by an unusual variant of pemphigus. He presented with a crusty and scaly lesion of the nose. We performed reflectance confocal microscopy and optical coherence tomography on the lesion, which suggested an unexpected diagnosis of pemphigus. Therefore, to confirm our diagnostic suspicions, we executed indirect immunofluorescence and two biopsies, one for histopathological examination and one for direct immunofluorescence. Histopathological evaluation showed acantholysis with formation of clefts in the granular and spinous layers of the epidermis. Direct immunofluorescence revealed immunoglobulin G and C3 deposit to the full thickness of the epidermis. Indirect immunofluorescence showed intercellular antibodies at a titer of 1:40 in the suprabasal epidermis. The immunoblot analysis using epidermal extract revealed the presence of circulating antibodies directed to 130- and 160-kDa antigens in the patient's serum. These two antigens were evidenced from nitrocellulose membrane with colorimetric AP systems, which highlighted the presence of autoantibodies against desmoglein (Dsg)1 and Dsg3 (sodium dodecylsulfate polyacrylamide gel electrophoresis). We also performed an enzyme-linked immunoassay. All these findings suggested that this patient's pemphigus had features of both vulgaris and foliaceus variants.
我们报告了一例74岁男性患有一种不寻常的天疱疮变体的病例。他的鼻子出现了结痂和鳞屑性病变。我们对该病变进行了反射共聚焦显微镜检查和光学相干断层扫描，结果提示为天疱疮这一意外诊断。因此，为了证实我们的诊断怀疑，我们进行了间接免疫荧光检查和两次活检，一次用于组织病理学检查，一次用于直接免疫荧光检查。组织病理学评估显示棘层松解，在表皮颗粒层和棘层形成裂隙。直接免疫荧光显示免疫球蛋白G和C3沉积于表皮全层。间接免疫荧光显示基底上层表皮细胞间抗体滴度为1:40。使用表皮提取物进行的免疫印迹分析显示患者血清中存在针对130 kDa和160 kDa抗原的循环抗体。通过比色碱性磷酸酶系统在硝酸纤维素膜上证实了这两种抗原，突出了抗桥粒芯糖蛋白（Dsg）1和Dsg3自身抗体的存在（十二烷基硫酸钠聚丙烯酰胺凝胶电泳）。我们还进行了酶联免疫吸附测定。所有这些发现表明该患者的天疱疮具有寻常型和落叶型变体的特征。
