增殖性脓性皮炎-脓性口炎

Pyodermatitis-pyostomatitis vegetans

患者女，42岁，因口唇脓疱性损害11个月，头面、躯干脓疱、溃疡、增殖性斑块5个月就诊。既往有溃疡性结肠炎病史8年。皮肤科情况：眼睑轻度糜烂，颊黏膜及口唇水肿，脓疱、渗出、黏液形成典型“蜗牛行迹”损害；颈部大片黑褐色增殖性斑块，约20cm×10cm，边缘隆起，分别于前胸、腋下、脐周、左侧腹股沟，可见手掌至核桃大小增殖性斑块，边缘脓性分泌物。组织病理：表皮呈乳头瘤样增生，表皮内可见嗜酸性粒细胞微脓疡，真皮浅层大量中性粒细胞、嗜酸性粒细胞浸润。直接免疫荧光检查IgA、IgG、C3阴性。诊断：增殖性脓性皮炎一脓性口炎。治疗：甲泼尼龙40mg／d静脉滴注及对症处理，皮疹逐渐消退。

A 42-year-old woman was admitted to the hospital for an ll-month history of pustular lesions of the mouth and lips as well as a 5-month history of pustules, ulcer, proliferative plaques on the head, neck and trunk. She had suffered from 8 years of ulcerative colitis, Dermatological examination revealed mild erosion of eyelids, swelling of buccal mueosa and oral lips, as well as typical ＂snail track＂ lesions formed by pustules, exu- dates and mucilage. There was a large pitchy proliferative plaque sized 20 cm x 10 cm with an elevated margin in the neck as well as palm- to persian walnut-sized proliferative plaques in the prothorax, armpit, periumbilical area and left inguinal area. Histopathology revealed epitheliomatous hyperplasia of the epidermis with the formation of eosinophilic mlcroabscesses, and superficial dermal infiltrate with numerous neutrophils and eosinophils. Direct immunofluorescence tests for IgA, IgG, and C3 were negative. A diagnosis of pyodermatitis-pyostomatitis vegetans was made. The patient was given intravenous methylprednisolone 40 mg/day and symptomatic treatment, which leaded to a gradual subsidence of skin lesions.