化脓性关节炎,痤疮,坏疽性脓皮病,化脓性汗腺炎(PAPASH);化脓性关节炎,坏疽性脓皮病(PG),和痤疮;PG,痤疮,化脓性汗腺炎;和PG,痤疮,脊椎关节炎(PASS)都是具有相似发病机制的自身炎症性疾病的一部分。它们与脯氨酸-丝氨酸-苏氨酸磷酸酶相互作用蛋白1中的各种突变有关,导致先天免疫系统的失调以及白细胞介素(IL)-1,IL-17和IL-23和肿瘤坏死因子(TNF)-α的过量产生。用生物制剂靶向这些细胞因子在治疗中起着重要作用。这里,我们正在描述一名患有PAPASH综合征的年轻男性,他接受了TNF-α和IL-1抑制剂的治疗。
Pyogenic arthritis, acne, pyoderma gangrenosum, and suppurative hidradenitis (PAPASH); pyogenic arthritis, pyoderma gangrenosum (PG), and acne; PG, acne, hidradenitis suppurativa; and PG, acne, spondylarthritis (PASS) are all part of a spectrum of autoinflammatory disorders that share similar pathogenesis. They are related to various mutations in the proline-serine-threonine phosphatase interacting protein 1, leading to dysregulation of the innate immune system and overproduction of interleukin (IL)-1, IL-17, and IL-23 and tumor necrosis factor (TNF)-α. Targeting these cytokines with biologics plays an important role in treatment. Here, we are describing the case of a young male with PAPASH syndrome who was treated with TNF-α and IL-1 inhibitor.