Shevtsova Inga N, Abbott James J, Shevtsov Pavel N, Bedoya Guiset Carvajal, Norton Diana C
Department of Internal Medicine, Billings Clinic, Billings, Montana, USA.
Department of Dermatology, Billings Clinic, Billings, Montana, USA.
Case Rep Rheumatol. 2025 Apr 22;2025:7720064. doi: 10.1155/crrh/7720064. eCollection 2025.
PAPASH syndrome, a rare autoinflammatory condition characterized by pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa, presents significant treatment challenges due to its rarity and complex multisystem involvement. Since its initial description in 2013, only 14 cases have been documented, leading to limited treatment experience. Although IL-1 and TNF-alpha blocking agents have shown efficacy, responses vary due to genetic and pathogenetic differences, with some cases being resistant. Therefore, alongside summarizing prior treatment experiences, new treatment modalities need to be explored. This report presents the case of a 46-year-old Native American male with PAPASH syndrome who responded successfully to IL-17 inhibition with secukinumab. The patient experienced marked improvement in both dermatologic and rheumatologic symptoms, highlighting the potential role of IL-17 in the pathogenesis of PAPASH. This case suggests that IL-17 inhibition could be a promising treatment modality for PAPASH syndrome.
PAPASH综合征是一种罕见的自身炎症性疾病，其特征为化脓性关节炎、坏疽性脓皮病、痤疮和化脓性汗腺炎，由于其罕见性和复杂的多系统受累，在治疗上面临重大挑战。自2013年首次描述以来，仅记录了14例病例，导致治疗经验有限。尽管白细胞介素-1（IL-1）和肿瘤坏死因子-α（TNF-α）阻断剂已显示出疗效，但由于基因和发病机制的差异，反应各不相同，有些病例具有耐药性。因此，在总结既往治疗经验的同时，需要探索新的治疗方式。本报告介绍了一名46岁患有PAPASH综合征的美国原住民男性病例，该患者使用司库奇尤单抗抑制IL-17后获得了成功治疗。患者的皮肤和风湿症状均有显著改善，突出了IL-17在PAPASH发病机制中的潜在作用。该病例表明，抑制IL-17可能是治疗PAPASH综合征的一种有前景的治疗方式。
