种痘样水疱病12例临床及病理分析

Clinical and pathological analysis of 12 children with hydroa vacciniforme

目的:分析12例种痘样水疱病患儿的临床、病理特征。方法:回顾分析12例种痘样水疱病患儿的临床资料和组织病理检查结果。结果:12例种痘样水疱病患儿男5例,女7例,平均发病年龄4.3岁,病程1～13年。12例均有暴露部位的丘疱疹、水疱、坏死、结痂及痘疮样瘢痕;其中1例伴有非暴露部位皮肤受累,1例伴有指间关节变形,1例皮损出现时伴有低热。病理表现为表皮内多房性水疱,真皮浅层中等至大量淋巴细胞浸润,并可伴有表皮、真皮坏死。结论:种痘样水疱病典型皮损为水疱、丘疱疹和痘疮样瘢痕,重型可出现皮肤肿胀,皮损亦可累及非暴露部位皮肤。

Objective: To analyse clinical and histophathological features of 12 cases of hydroa vacciniforme (HV). Methods: Twelve patients with HV from Beijing Childrens Hospital were enrolled in this study. The clinical manifestations and paraffin-embedded biopsy specimens of the 12 cases were examined and analysed retrospectively. Results: Among the 12 patients, 7 were female and 5 were male. The mean age of onset was 4.3 years old (ranging from 2 to 7 years old), the duration of the disease was 1 to 13 years. All patients presented papulo-vesicular, vesicular, necrotic, crusted, and vacciniform scar on the exposed areas. Of these patients, one case had the involvement on unexposed skin, one case with the malformation of interphalangeal joints, and one case with mild fever. Histologically, there were intra-epidermal multi-locular vesicles with dense lymphocytic infiltration in the superficial dermis, and necrosis of epidermis and dermis. Conclusions: The typical skin eruptions of HV include vesicules, vesiculopapules and vacciniform scarring. There may be edema of the skin and involvement of skin lesions in unexposed area.