Morphea is an autoimmune fibrotic skin disease. Eosinophilic fasciitis (EF) is considered to belong to the severe spectrum of morphea. We conducted a scoping review assessing the risk of secondary cancer among morphea/EF patients, paraneoplastic morphea/EF and morphea/EF developing secondary to cancer therapy. The search was conducted using MEDLINE, Embase, Cochrane databases for articles published from inception to September 2022 following the Preferred Reporting Items for Systematic reviews and Meta-Analyses for Scoping Reviews (PRISMA-ScR) guidelines with no language or date restrictions. Two hundred and one studies were included. Of these, 32 studies reported on secondary cancer in morphea/EF patients, 45 on paraneoplastic morphea/EF and 125 on cancer-treatment-induced morphea/EF. While the current evidence remains limited, data suggest an increased risk of secondary cutaneous and possibly pancreatic malignancy in morphea patients, particularly the generalized subtype. There were insufficient data for EF. On the other hand, paraneoplastic morphea was anecdotal, whereas several observational studies suggested that ~10% of EF cases may be paraneoplastic, primarily in the context of hematologic malignancies. Radiotherapy-induced morphea is rare, seen in ~0.2% of treated patients and is usually localized to the treatment site, except in patients with pre-existing autoimmunity. While chemotherapy-induced cases are reported, immunotherapy morphea/EF cases are emerging and are preferentially seen with PD-1 and not CTLA-4 inhibitors. This study is limited by the type of articles included (case reports, case series and observational studies), and hence, additional research on this important topic is needed.
硬斑病是一种自身免疫性纤维化皮肤病。嗜酸性筋膜炎被认为是硬斑病的严重谱系表现。本研究通过范围综述评估了硬斑病/嗜酸性筋膜炎患者继发癌症的风险、副肿瘤性硬斑病/嗜酸性筋膜炎以及癌症治疗后继发的硬斑病/嗜酸性筋膜炎。检索遵循系统综述和荟萃分析范围综述报告规范指南,在MEDLINE、Embase和Cochrane数据库中检索建库至2022年9月发表的相关文献,无语言或日期限制。共纳入201项研究,其中32项研究报告了硬斑病/嗜酸性筋膜炎患者的继发癌症情况,45项涉及副肿瘤性硬斑病/嗜酸性筋膜炎,125项关注癌症治疗诱发的硬斑病/嗜酸性筋膜炎。现有证据虽有限,但数据表明硬斑病患者(尤其是泛发性亚型)继发皮肤恶性肿瘤及可能胰腺恶性肿瘤的风险增加,嗜酸性筋膜炎的相关数据尚不充分。副肿瘤性硬斑病多为个案报道,而多项观察性研究表明约10%的嗜酸性筋膜炎病例可能具有副肿瘤性,主要与血液系统恶性肿瘤相关。放疗诱发的硬斑病较为罕见(约0.2%的接受治疗者),通常局限于治疗部位,但既往存在自身免疫性疾病的患者例外。化疗诱发病例虽有报道,但免疫治疗相关的硬斑病/嗜酸性筋膜炎病例正逐渐显现,且多见于PD-1抑制剂治疗而非CTLA-4抑制剂。本研究受限于纳入文献类型(病例报告、病例系列和观察性研究),因此该重要议题仍需进一步深入研究。
Morphea, Eosinophilic Fasciitis and Cancer: A Scoping Review