Fischer Alexander H, Jourabchi Natanel, Khalifian Saami, Lazarus Gerald S
Department of Dermatology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Department of Dermatology, Harvard Medical School, Boston, Massachusetts, USA.
Wound Repair Regen. 2022 May;30(3):338-344. doi: 10.1111/wrr.13014. Epub 2022 Apr 27.
Pyoderma gangrenosum (PG) has been linked to various underlying systemic diseases; many associations are based on case reports or small case series, including hidradenitis suppurativa. Literature examining systemic therapies according to underlying comorbid condition is limited. The study objective was to investigate comorbid diseases of PG and correlate disease associations with effectiveness of therapeutic interventions. Using Johns Hopkins Medical Institutions medical records, 220 patients had an ICD-9 code of 686.01 for PG between 1 January 2006 and 30 June 2015, of whom 130 patients met rigorous inclusion/exclusion criteria for PG (non-peristomal). The 130 PG patients in our study were 69% female, 58% Caucasian, and 35% African American. Documented comorbid conditions included inflammatory bowel disease (IBD; 35%), rheumatoid arthritis (RA; 12%), hidradenitis suppurativa (HS; 14%), and monoclonal gammopathy (12%). PG patients with HS versus without HS were more likely to be African-American (83% vs. 28%; P < 0.001) and had an earlier mean age of PG onset (38 vs. 48 years; P = 0.02). Strikingly, 53% of female African-American patients with PG onset prior to age 40 had comorbid HS. Comorbid inflammatory bowel disease was observed in 38% of PG patients with RA, 28% of PG patients with HS, and 27% of PG patients with monoclonal gammopathy. Of the 32 patients who received infliximab for active PG, complete ulcer healing was observed in 83% (5/6) of patients with comorbid HS versus 31% (8/26) of patients without HS (Fisher exact P = 0.03). Screening patients for associated systemic disease for multiple related illnesses is essential. Effectiveness of systemic therapy may depend upon the underlying systemic disease; hidradenitis suppurativa may be a specific example.
坏疽性脓皮病（PG）与多种潜在的全身性疾病有关；许多关联是基于病例报告或小病例系列，包括化脓性汗腺炎。根据潜在合并症研究全身治疗方法的文献有限。本研究的目的是调查PG的合并疾病，并将疾病关联与治疗干预的有效性相关联。利用约翰霍普金斯医疗机构的医疗记录，在2006年1月1日至2015年6月30日期间，220例患者的PG国际疾病分类第九版代码为686.01，其中130例患者符合PG（非造口周围）的严格纳入/排除标准。我们研究中的130例PG患者中，69%为女性，58%为白种人，35%为非裔美国人。记录的合并症包括炎症性肠病（IBD；35%）、类风湿性关节炎（RA；12%）、化脓性汗腺炎（HS；14%）和单克隆丙种球蛋白病（12%）。患有HS的PG患者与未患有HS的患者相比，更可能是非裔美国人（83%对28%；P < 0.001），且PG发病的平均年龄更早（38岁对48岁；P = 0.02）。引人注目的是，40岁之前发病的女性非裔美国PG患者中有53%患有合并HS。在患有RA的PG患者中，38%观察到合并炎症性肠病；在患有HS的PG患者中，28%观察到合并炎症性肠病；在患有单克隆丙种球蛋白病的PG患者中，27%观察到合并炎症性肠病。在32例因活动性PG接受英夫利昔单抗治疗的患者中，患有合并HS的患者中有83%（5/6）实现了溃疡完全愈合，而未患有HS的患者中这一比例为31%（8/26）（Fisher精确检验P = 0.03）。筛查患者是否患有多种相关疾病的相关全身性疾病至关重要。全身治疗的有效性可能取决于潜在的全身性疾病；化脓性汗腺炎可能就是一个具体例子。
