Department of Dermatology, University of California, San Francisco, San Francisco, California.
Department of Dermatology, Virginia Commonwealth University, Richmond, Virginia.
J Am Acad Dermatol. 2018 Jun;78(6):1195-1204.e1. doi: 10.1016/j.jaad.2017.12.049. Epub 2017 Dec 27.
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.
We sought to identify important clinical features of PPG and effective treatments available for its management.
A systematic literature review of PPG was performed using PubMed, Medline, and Embase databases.
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.
PPG is a rare disease lacking randomized trials or diagnostic guidelines. Treatment duration and follow-up time among studies are variable.
Key clinical characteristics of PPG are highlighted. Several treatments, including a more prominent role for surgical intervention, may be effective for PPG treatment.
肠造口周围坏疽性脓皮病(PPG)是坏疽性脓皮病的一种罕见亚型。PPG 的诊断和治疗具有挑战性;目前尚无循证指南。
我们旨在确定 PPG 的重要临床特征和有效的治疗方法。
使用 PubMed、Medline 和 Embase 数据库对 PPG 进行系统的文献回顾。
我们描述了来自 79 项研究的 335 例 PPG 患者。临床特征包括疼痛、迅速进展的溃疡,边缘有侵蚀性、紫蓝色,有造口漏和局部皮肤刺激或创伤史。皮质类固醇是一线治疗;英夫利昔单抗和阿达木单抗可同时控制活动性炎症性肠病。局部和全身联合治疗通常被使用。伤口敷料、载体选择以及适当的造口装置以尽量减少漏液、刺激和压力引起的缺血可以改善愈合。与经典溃疡性坏疽性脓皮病不同,手术方法,如造口关闭和切除活动性炎症性肠病,在 PPG 管理中具有有效的作用。
PPG 是一种罕见疾病,缺乏随机试验或诊断指南。研究中的治疗持续时间和随访时间各不相同。
突出了 PPG 的关键临床特征。一些治疗方法,包括更突出的手术干预作用,可能对 PPG 的治疗有效。