坏疽性脓皮病36例临床特点分析及PARACELSUS评分与Delphi标准的比较  

Analysis of clinical characteristics of 36 patients with pyoderma gangrenosum and comparison between the PARACELSUS score and Delphi criteria for their diagnosis

作　　者：胡清洁 周敏 姚煦 Hu Qingjie;Zhou Min;Yao Xu(Department of Allergy and Rheumatology,Hospital of Dermatology,Chinese Academy of Medical Sciences and Peking Union Medical College,Nanjing 210042,China)

机构地区：[1]中国医学科学院、北京协和医学院皮肤病医院过敏与风湿免疫科,南京210042

出　　处：《中华皮肤科杂志》2023年第5期410-414,共5页Chinese Journal of Dermatology

摘　　要：目的分析36例坏疽性脓皮病患者的临床特点及治疗转归,比较和评价PARACELSUS评分和Delphi标准的适用性和一致性。方法收集2000年1月至2022年1月在中国医学科学院皮肤病医院住院确诊为坏疽性脓皮病患者36例的临床资料。将PARACELSUS评分和Delphi标准分别应用于上述患者的诊断,并根据kappa值评估两种诊断方法的一致性。结果36例患者中,6例(16.67%)发病前有明确的诱发因素,31例(86.11%)自觉皮疹不同程度疼痛。31例(86.11%)皮疹形态以溃疡型为主,主要累及双下肢,16例(44.44%)为全身多部位受累。4例(11.11%)合并炎症性肠病,3例(8.33%)合并炎症性关节炎。系统用药以糖皮质激素、雷公藤和环孢素为主,8例(22.22%)使用了肿瘤坏死因子α拮抗剂。用PARACELSUS评分诊断22例(64.71%),用Delphi标准诊断17例(50.00%)。kappa一致性检验显示,两种诊断标准的一致性为中等,kappa值为0.47(95%CI:0.19~0.76,P=0.004)。结论坏疽性脓皮病的临床表现以经典溃疡型为主,常伴有炎症性肠病和炎症性关节炎,糖皮质激素和免疫抑制剂为主要治疗药物。PARACELSUS评分和Delphi标准侧重于坏疽性脓皮病的不同方面,在缺乏典型病理结果的情况下,推荐使用PARACELSUS评分诊断坏疽性脓皮病。Objective To analyze clinical characteristics,treatment,and outcomes of 36 patients with pyoderma gangrenosum,and to compare and evaluate the applicability and consistency between the PARACELSUS score and Delphi criteria.Methods From January 2000 to January 2022,clinical data were collected from 36 patients who were diagnosed with pyoderma gangrenosum in the Hospital of Dermatology,Chinese Academy of Medical Sciences.The PARACELSUS score and Delphi criteria were applied to their diagnosis,and the kappa test was used to evaluate the consistency between the two diagnostic criteria.Results Among the 36 patients,6(16.67%)had definite precipitating factors before the onset,and 31(86.11%)exhibited lesions with different degrees of pain.Ulcerative lesions predominatd in 31(86.11%)patients,which mostly involved the lower extremities,while 16(44.44%)presented with multiple lesions all over the body.Four(11.11%)patients were complicated by inflammatory bowel disease,and 3(8.33%)with inflammatory arthritis.Glucocorticoids,Tripterygium wilfordii,and cyclosporin were the main systemic treatment options,and tumor necrosis factor-αantagonist was used in 8(22.22%)patients.Twenty-two(64.71%)and 17(50.00%)patients were diagnosed with pyoderma gangrenosum using the PARACELSUS score and Delphi criteria,respectively.The kappa test showed moderate agreement between the two diagnostic criteria(kappa value=0.47,95%CI:0.19-0.76,P=0.004).Conclusions Classic ulcerative subtype was the major subtype in the patients with pyoderma gangrenosum,who were usually complicated by inflammatory bowel disease and inflammatory arthritis.Glucocorticoids and immunosuppressants were the main therapeutic drugs.The PARACELSUS score and Delphi criteria focus on different aspects of pyoderma gangrenosum,and the PARACELSUS score is recommended in the context of absence of typical histopathological manifestations.

关 键 词：坏疽性脓皮病 诊断 PARACELSUS评分 Delphi标准 一致性 

分 类 号：R753.6[医药卫生—皮肤病学与性病学]