Shaigany Sheila, Wong Priscilla W, Caplan Avrom, Kim Randie H, Femia Alisa
Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, New Hyde Park, New York, USA.
The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, 240 East 38th St, 11th Fl, New York, NY, 10016, USA.
Arch Dermatol Res. 2023 Jan;315(1):95-99. doi: 10.1007/s00403-021-02278-z. Epub 2021 Oct 29.
Pyoderma gangrenosum (PG) is a rare, and often challenging to diagnose, inflammatory disorder with relatively high rates of morbidity and mortality. Central to the diagnosis of PG is histologic evaluation and exclusion of other entities. Large-scale studies investigating the proportion of patients receiving a thorough diagnostic work-up, as well as prevalence studies regarding comorbidities and systemic treatment in PG using claims-based data, are sparse. Our objective was to identify patients diagnosed with PG and describe the diagnostic work-up and prevalence of common comorbidities and therapies in this population using claims-based data in a retrospective cohort study. In order to better understand practices of diagnostic work-up, we captured rates of skin biopsy, tissue culture, and/or surgical debridement prior to initial diagnosis. We also identified the prevalence of PG-associated comorbidities and initial immunosuppressive therapy given for PG. Of the 565 patients diagnosed with PG, 9.4% underwent skin biopsy, 8% tissue culture, and 1.4% both skin biopsy AND tissue culture prior to diagnosis. Inflammatory bowel disease was the most prevalent comorbidity (16.3%). The most common treatment administered was systemic corticosteroids (17%). Although practice guidelines explicitly delineate histology and exclusion of infection as important diagnostic criteria, only a minority of patients in this study underwent skin biopsy and/or tissue culture prior to receiving a diagnosis of PG, suggesting that patients may receive a diagnosis of PG without having tissue evaluation. Such discordance between practice guidelines and "real-world" practice inevitably increases the risk for misdiagnosis of PG and misdirected treatment with immunosuppressants for presumptive PG in cases of PG mimickers. Moreover, comorbidities associated with PG may occur, or be identified in, a lower proportion of patients as compared with what is reported in the existing literature. Study limitations include a population restricted to < 65 years with commercial insurance and the reliance upon ICD diagnostic coding to capture the population.
坏疽性脓皮病（PG）是一种罕见的炎症性疾病，诊断往往具有挑战性，发病率和死亡率相对较高。PG诊断的核心是组织学评估以及排除其他疾病。关于接受全面诊断检查的患者比例的大规模研究，以及使用基于索赔数据的PG合并症和全身治疗的患病率研究都很稀少。我们的目标是在一项回顾性队列研究中，使用基于索赔的数据识别诊断为PG的患者，并描述该人群的诊断检查、常见合并症和治疗方法的患病率。为了更好地了解诊断检查的做法，我们记录了初诊前皮肤活检、组织培养和/或手术清创的比例。我们还确定了PG相关合并症的患病率以及针对PG给予的初始免疫抑制治疗。在565例诊断为PG的患者中，9.4%在诊断前接受了皮肤活检，8%进行了组织培养，1.4%同时进行了皮肤活检和组织培养。炎症性肠病是最常见的合并症（16.3%）。最常用的治疗方法是全身使用皮质类固醇（17%）。尽管实践指南明确将组织学和排除感染作为重要的诊断标准，但在本研究中，只有少数患者在诊断为PG之前接受了皮肤活检和/或组织培养，这表明患者可能在没有组织评估的情况下被诊断为PG。实践指南与“现实世界”实践之间的这种不一致不可避免地增加了PG误诊的风险，以及在PG模仿者病例中对疑似PG错误使用免疫抑制剂治疗的风险。此外，与现有文献报道相比，与PG相关的合并症在患者中出现或被识别的比例可能较低。研究局限性包括研究人群限于年龄小于65岁且有商业保险的人群，以及依赖国际疾病分类（ICD）诊断编码来确定研究人群。
