61例儿童脓疱型银屑病临床分析

目的：探讨儿童脓疱型银屑病(pustular psoriasis, PP)及各亚型的临床特征及治疗方案。方法：回顾性分析2013年12月至2018年6月在我院住院治疗的61例儿童PP患者的临床资料。 结果：本组资料共包括61例儿童PP，分为四个亚型：泛发型(generalized pustular psoriasis, GPP; von Zumbusch)35例，环形型(annular pustular psoriasis, APP)4例，寻常型银屑病伴脓疱型(psoriasis vulgaris with pustules, PVP)15例和红皮病型脓疱型银屑病(pustular erythrodermic psoriasis, PEP)7例。上呼吸道感染和药物停用是最常见的诱发因素。von Zumbusch发病年龄最小(8.0岁)，APP发病年龄最大(12岁)。除了PVP（0.67∶1），其余各型男童比例均高于女童，且PVP中合并PV和有银屑病家族史比例明显高于其他类型，分别为73.3%和40.0%。相反，von Zumbusch有银屑病家族史的比例最小(11.4%)，von Zumbusch和APP的主要治疗方案为一线治疗药物单一疗法联合基础治疗，阿维A是主要的治疗药物。基础治疗是PVP的主要治疗方案。PEP病情严重，需阿维A联合甲氨蝶呤和基础治疗药物。结论：儿童PP各亚型的临床特征及治疗方案存在差异，正确的分型有助于选择合理的治疗方案。

Clinical analysis of 61 childhood pustular psoriasis

Objective: To investigate the clinical features and treatments of childhood pustular psoriasis (PP) and subtypes. Methods: A retrospective study was undertaken for 61 inpatient childhood PP in our hospital from Dec.2013 to Jul. 2018. Results: Totally 61 patients with  childhood PP  were analysed including35 cases of generalized pustular psoriasis (GPP; von Zumbusch), 4 cases of annular pustular psoriasis (APP), 15 cases of psoriasis vulgaris with pustules (PVP) and 7 cases of pustular erythrodermic psoriasis (PEP). Upper respiratory tract infections and drug withdrawal were the most common predisposing factors. The median age of onset was smallest (8.0 years old) in von Zumbusch and largest (12 years old) in APP. The proportion of boys was higher than girls in all variants except PVP (0.67∶1), and the proportion of patients with previous PV (73.3%) and family history (40.0%) was higher in PVP than others. In contrast, there was the smallest rate for patients with family history (11.4%) in von Zumbusch. The main treatments for von Zumbusch and APP were first-line monotherapy combined with basic treatments, and acitretin was the main choice. Basic treatments were the main treatments for PVP. PEP was a serious subtype which needed acitretin to combine with methotrexate and basic treatments. Conclusion: The clinical characteristics and treatments are different between subtypes of childhood PP, and correct typing contributes to choosing reasonable treatments.