Pyoderma Gangrenosum A Comparison of Typical and Atypical Forms with an Emphasis on Time to Remission. Case Review of 86 Patients from 2 Institutions
Pyoderma Gangrenosum A Comparison of Typical and Atypical Forms with an Emphasis on Time to Remission. Case Review of 86 Patients from 2 Institutions
复制标题
坏疽脓皮病典型和非典型形式的比较，重点是缓解时间。
作者：
M. L. Bennett;J. Jackson;J. Jorizzo;A. Fleischer;W. White;J. Callen
坏疽脓皮病是一种病因不明的特发性、炎症性、溃疡性疾病。诊断基于临床和病理特征，需要排除产生溃疡的条件。存在一种非典型大疱性变异型(非典型坏疽脓皮病，APG)，其临床特征与Sweet综合征相似。由于PG是一种罕见的疾病，很少有大型病例系列的报道。坏疽脓皮病在20世纪上半叶首次被认为是一种独特的疾病实体。对PG的累积知识是基于少数病例系列和多个个别病例报告。为了增加这方面的知识，我们在相当长的一段时间内展示了我们与大量患者的经验。我们对86例PG患者的病历进行了回顾分析，这些患者在两所大学的皮肤科接受了12年的评估和治疗。PG和APG的平均发病年龄(+/-标准差)分别为44.6±19.7岁和52.2±15.3岁。PG以下肢受累最多见，APG以上肢受累最多见。50%的患者出现相关的全身疾病。炎症性肠病在PG患者中最常见，而血液病或恶性肿瘤在APG患者中最常见。虽然少数患者接受了局部治疗或非免疫抑制治疗，但大多数患者需要口服皮质类固醇治疗，通常是全身免疫抑制治疗。PG患者平均需要11.5+/-11.1个月的治疗才能获得缓解，而APG患者的平均治疗需要9.0+/-13.7个月。5名患者(5.8%)患有对多种强化治疗极其难治的疾病。PG和APG的预后和疾病相关性似乎是不同的。与PG相比，APG更常与血液病或恶性肿瘤相关，且起效更快。
Pyoderma gangrenosum (PG) is an idiopathic, inflammatory, ulcerative disease of undetermined cause. The diagnosis is based on clinical and pathologic features and requires exclusion of conditions that produce ulcerations. An atypical bullous variant (atypical pyoderma gangrenosum, APG) exists with clinical features similar to those of Sweet syndrome. Because PG is a rare disease, few large case series have been reported. Pyoderma gangrenosum was first recognized as a unique disease entity in the first half of the 20th century. Cumulative knowledge of PG is based on a handful of case series and multiple individual case reports. To augment that knowledge, we present our experience with a large number of patients over a significant time. We performed a retrospective analysis of the medical records of 86 patients with PG who were evaluated and treated over 12 years at 2 university-based dermatology departments. The mean (+/- standard deviation) age of onset of PG and APG, respectively, was 44.6 +/- 19.7 years and 52.2 +/- 15.3 years. Lower extremity involvement was most common in PG, whereas upper extremity involvement was most common in APG. Associated relevant systemic diseases were seen in 50% of patients. Inflammatory bowel disease was the most common association in patients with PG, whereas hematologic disease or malignancy was most common in those with APG. Although a few patients were managed with local measures or nonimmunosuppressive treatment, the majority required oral corticosteroid therapy, often with systemic immunosuppressive treatment. PG patients required a mean 11.5 +/- 11.1 months of treatment to achieve remission compared with 9.0 +/- 13.7 months for patients with APG. Five patients (5.8%) had disease that was extremely refractory to multiple intensive therapies. The prognosis and disease associations for PG and APG appear to be different. Compared with PG, APG is more often associated with hematologic disease or malignancy, and remits more quickly.
静脉注射甲泼尼龙后出现过敏反应。
DOI：
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发表时间：
1981
期刊：
JAMA : the journal of the American Medical Association
影响因子：
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作者：
Freedman,MD;Schocket,AL;Chapel,N;Gerber,JG
通讯作者：
Gerber,JG