Department of Dermatology, Mayo Clinic, Rochester, MN, Canada.
Department of Hematology, Mayo Clinic, Rochester, MN, USA.
Arch Dermatol Res. 2024 May 25;316(6):268. doi: 10.1007/s00403-024-03098-7.
Pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterized by ulcerative painful lesions with violaceous undermined borders. Up to 75% of PG cases develop in association with an underlying systemic disease. Monoclonal gammopathy is reportedly a concomitant condition with PG, with studies indicating immunoglobulin (Ig) A gammopathy as the most common. Whether gammopathy is associated with PG or is an incidental finding has been debated. We sought to investigate the association and characteristics of gammopathy in patients with PG. We retrospectively identified PG patients at our institution from 2010 to 2022 who were screened for plasma cell dyscrasia. Of 106 patients identified, 29 (27%) had a gammopathy; subtypes included IgA (41%), IgG (28%), and biclonal (IgA and IgG) (14%). Mean age was similar between those with and without gammopathy (60.7 vs. 55.9 years; P = .26). In addition, hematologic or solid organ cancer developed in significantly more patients with vs. without gammopathy (8/29 [28%] vs. 5/77 [6%]; P = .003). Among the subtypes of gammopathy, IgG monoclonal gammopathy had the highest proportion of patients with subsequent cancer development (4 of 8 patients, 50%). Study limitations include a retrospective, single-institution design with a limited number of patients. Overall, our data show a high prevalence of gammopathy in patients with PG; those patients additionally had an increased incidence of cancer, especially hematologic cancer.
坏疽性脓皮病(PG)是一种以溃疡性疼痛性皮损为特征的中性粒细胞皮肤病,其边缘呈暗紫色潜行状。高达 75%的 PG 病例与潜在的系统性疾病有关。据报道,单克隆丙种球蛋白病是 PG 的伴随病症,研究表明 IgA 丙种球蛋白病最为常见。丙种球蛋白病是否与 PG 相关,还是偶然发现,一直存在争议。我们试图研究 PG 患者丙种球蛋白病的相关性和特征。我们对 2010 年至 2022 年我院收治的 PG 患者进行了回顾性研究,对这些患者进行了浆细胞异常的筛查。在 106 名患者中,有 29 名(27%)患有丙种球蛋白病;亚类包括 IgA(41%)、IgG(28%)和双克隆(IgA 和 IgG)(14%)。有和无丙种球蛋白病患者的平均年龄相似(60.7 岁 vs. 55.9 岁;P=0.26)。此外,有丙种球蛋白病的患者比无丙种球蛋白病的患者更易发生血液系统或实体器官癌症(29/87 [28%] vs. 7/99 [6%];P=0.003)。在丙种球蛋白病的亚型中,IgG 单克隆丙种球蛋白病患者发生后续癌症的比例最高(8 例患者中有 4 例,50%)。研究的局限性包括回顾性、单机构设计和患者数量有限。总的来说,我们的数据显示 PG 患者丙种球蛋白病的患病率较高;这些患者的癌症发病率更高,尤其是血液系统癌症。