Background

Peristomal pyoderma gangrenosum (PPG) is an uncommon subtype of pyoderma gangrenosum. PPG is a challenging condition to diagnose and treat; no evidence-based guidelines exist.

Objective

We sought to identify important clinical features of PPG and effective treatments available for its management.

Methods

A systematic literature review of PPG was performed using PubMed, Medline, and Embase databases.

Results

We describe 335 patients with PPG from 79 studies. Clinical features include a painful, rapidly progressing ulcer with undermined, violaceous borders with a history of ostomy leakage and local skin irritation or trauma. Systemic steroids are first-line therapy; infliximab and adalimumab provide concomitant control of active inflammatory bowel disease. Combination local and systemic therapy was commonly used. Wound dressings, vehicle selection, and appropriate ostomy devices to minimize leakage, irritation, and pressure-induced ischemia can improve healing. Distinct from classic ulcerative pyoderma gangrenosum, surgical approaches, such as stoma closure and resection of active inflammatory bowel disease, have an effective role in PPG management.

Limitations

PPG is a rare disease lacking randomized trials or diagnostic guidelines. Treatment duration and follow-up time among studies are variable.

Conclusions

Key clinical characteristics of PPG are highlighted. Several treatments, including a more prominent role for surgical intervention, may be effective for PPG treatment.

中文翻译：

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坏死性脓皮性脓性脓皮病的诊断和处理：系统评价

背景

坏疽性脓皮病性脓皮病（PPG）是坏疽性脓皮病的一种亚型。PPG是诊断和治疗的挑战性疾病。没有基于证据的指南。

客观的

我们试图确定PPG的重要临床特征以及可用于其管理的有效治疗方法。

方法

使用PubMed，Medline和Embase数据库对PPG进行了系统的文献综述。

结果

我们从79项研究中描述了335例PPG患者。临床特征包括疼痛迅速发展的溃疡，边界破损，紫胶，有造口术渗漏和局部皮肤刺激或外伤史。全身性类固醇是一线治疗；英夫利昔单抗和阿达木单抗可同时控制活跃的炎症性肠病。局部和全身治疗相结合是常用的方法。伤口敷料，媒介物选择和适当的造口术设备可最大程度地减少渗漏，刺激和压力引起的局部缺血，可改善愈合效果。与典型的溃疡性坏疽性脓皮病不同，手术方法（如造口关闭和活动性炎症性肠病切除）在PPG管理中具有有效作用。

局限性

PPG是一种罕见的疾病，缺乏随机试验或诊断指南。研究之间的治疗持续时间和随访时间是可变的。

结论

PPG的关键临床特征被强调。PPG治疗可能有效的几种治疗方法，包括在外科手术中发挥更重要的作用。