儿童泛发性脓疱型银屑病32例临床与病理分析

The clinical and pathological analysis of 32 children with Zumbusch pattern of generalized pustular psoriasis

目的探讨儿童泛发性脓疱型银屑病的发病及治疗情况,以提高治愈率。方法对32例儿童泛发性脓疱型银屑病的临床症状及病理检查进行分析。结果32例患儿均有其典型的临床表现,男女发病之比为2.2∶1,平均年龄6.45岁,取新脓疱作病理活检,发现角质形成细胞变性,表皮水肿,表皮内有中性粒细胞移入,棘层上部海绵样脓疱形成,真皮血管扩张,血管周围淋巴细胞及中性粒细胞浸润。结论儿童泛发性脓疱型银屑病为一种少见的疾病,临床及组织病理具有特征性。治疗应去除诱发因素,及时控制感染,可在实验室定期监控下,首选新体卡松口服,症状可得到明显改善。

Objectives To improve the cure rate by investigating the clinical features and treatment of children with Zumbusch pattern of generalized pustular psoriasis. Methods The study recruited 32 children with typical clinical manifestations. The male to female ratio was 2.2 ： 1 with an average age of 6.45 years. Biopsy was performed on the children who developed new pustules. Results Histopathological analysis showed degenerated keratinocytes, edema and neutrophilic infiltration of epithelium, spongiform pustules on the upper acanthoid stratum, dilated dermal vessels and perivascular lymphocytic and neutrophillic infiltration. Conclusions Childhood Zumbusch pattern of generalized pustular psoriasis is a rare disease with typical clinical and pathological features. Neotigason can effectively relieve the symptoms. It can be administrated as the first-line of treatment with regular laboratory tests after the elimination of the triggering factors and the control of infection.