Department of Dermatology and Allergy, Technical University of Munich, Munich, Germany.
Department of Dermatology, Hamamatsu University School of Medicine, Hamamatsu, Japan.
J Eur Acad Dermatol Venereol. 2022 Sep;36(9):1501-1506. doi: 10.1111/jdv.18172. Epub 2022 May 12.
In the late 1960s, palmoplantar pustulosis (PPP) with sternocostoclavicular arthropathy was first described in Japan, predominantly affecting women in the perimenopausal age. In the 1970s, the chronic non-bacterial osteomyelitis and chronic recurrent multifocal osteomyelitis were initially observed in paediatric patients with approximately 70% girls. Acne fulminans accompanied by polyarthralgia have been observed since early 1970s, which almost exclusively occurs in adolescent boys. Report on spondyloarthropathy associated with hidradenitis suppurativa can be traced back to 1982. The SAPHO syndrome was coined in 1987 to lump together synovitis, acne, pustulosis, hyperostosis and osteitis to conceptualize a group of inflammatory osteocutaneous diseases of unclear etiopathogenesis and ill-defined associations spanning disparate age and gender groups. From historical view, Sasaki syndrome is proposed to replace SAPHO syndrome to represent PPP with sternocostoclavicular arthropathy in the absence of other skin manifestations. Hidradenitis suppurativa is folliculitis in pathogenesis and no longer classified as acne. PPP accompanied by psoriasis vulgaris is more likely psoriasis pustulosa palmoplantaris in dermatological aspect, and the associated arthritis is part of psoriatic arthropathy. Pathophysiology of these disorders is incompletely understood. To echo the advancement of high-throughput sequencing, splitting but not lumping of clinical findings would be a better strategy to decipher these multigenic complex inflammatory disorders.
二十世纪六十年代末,日本首次描述了掌跖脓疱病伴胸锁关节炎(PPP),主要影响围绝经期妇女。二十世纪七十年代,慢性非细菌性骨髓炎和慢性复发性多灶性骨髓炎最初在儿童患者中观察到,约 70%为女孩。自二十世纪七十年代初以来,人们观察到伴有多关节炎的暴发性痤疮,几乎仅发生于青少年男孩。与化脓性汗腺炎相关的脊柱关节病的报告可以追溯到 1982 年。SAPHO 综合征于 1987 年被提出,用于将滑膜炎、痤疮、脓疱病、骨肥厚和骨炎汇集在一起,以概念化一组发病机制不明和关联不明确的炎症性骨皮病,涉及不同年龄和性别群体。从历史上看,Sasaki 综合征被提议取代 SAPHO 综合征,以代表缺乏其他皮肤表现的掌跖脓疱病伴胸锁关节炎。化脓性汗腺炎在发病机制上是毛囊炎,不再被归类为痤疮。伴有寻常型银屑病的掌跖脓疱病在皮肤科方面更可能是掌跖脓疱性银屑病,相关关节炎是银屑病关节炎的一部分。这些疾病的发病机制尚不完全清楚。为了呼应高通量测序的进展,对临床发现进行拆分而不是合并将是破译这些多基因复杂炎症性疾病的更好策略。