连续性肢端皮炎样增生型天疱疮

Pemphigus vegetans presenting as acrodermatitis continua: a case report

报告1例发生于指趾类似于连续性肢端皮炎的增殖型天疱疮。患者男,70岁。唇部、口腔黏膜出现脓疱并增生8个月,指、趾脓疱增生1个半月。皮损组织病理检查示表皮呈假性上皮瘤样增生,基底层上方棘层松解,角质层中性粒细胞和嗜酸性粒细胞移入,可见嗜酸性脓疱形成。直接免疫荧光检查示棘细胞间近基底层IgG和C3呈网状沉积。间接免疫荧光检查示棘细胞间偏下方IgG呈网状沉积,滴度为1∶10。给予中等剂量糖皮质激素治疗后皮损很快好转。

A case with clinical signs of acrodermatitis continua of digits and toes was presented. A 70-year-old man had pustules and vegetations on his lips and buccal mucosa for 8 months, pustules and vegetations on digits and toes for one and a half months. Histopathologic findings showed pseudoepitheliomatous hyperplasia of epithelium, suprabasilar acantholysis and intraepidermal abscesses filled with eosinophils. Direct immunofluorescence examination was positive for IgG and C3 in the intercellular regions of the lowermost epidermis. Indirect immunofluorescence showed intercellular deposition of IgG at a titer of 1：10. He was treated successfully with medium-dose glucocorticoid therapy.