坏疽性脓皮病,痤疮,化脓性汗腺炎综合征是一种罕见的以坏疽性脓皮病（PG）为特征的炎症性疾病，轻度至重度面部痤疮，和化脓性汗腺炎（HS）。它仅影响皮肤并代表一系列自身炎症的皮肤特征。化脓性无菌性关节炎（PA）的缺乏区分坏疽性脓皮病，痤疮,化脓性关节炎引起的化脓性汗腺炎（PASH）综合征，坏疽性脓皮病,痤疮,和化脓性汗腺炎（PA-PASH），坏疽性脓皮病,痤疮,化脓性汗腺炎,强直性脊柱炎（PASS），和化脓性关节炎，坏疽性脓皮病,和痤疮（PAPA）综合征。PASH综合征的确切病因和发病机制尚不清楚。PG和HS都包含在中性粒细胞性皮炎的范围内，这被认为是一种自身炎症综合征。从病理生理学的角度来看，它们表现出相似的机制，包括富含中性粒细胞的皮肤浸润和白细胞介素-1（IL-1）家族的过度表达。这些发现为这些顽固性疾病提供了指导。在这次审查中,我们描述了1例PASH综合征患者,该患者最初对免疫抑制治疗无反应,但对秋水仙碱和沙利度胺联合治疗有反应.我们回顾了有关PASH综合征管理的相关文献。
Pyoderma gangrenosum, acne, and hidradenitis suppurativa syndrome is a rare inflammatory disease characterized by pyoderma gangrenosum (PG), mild to severe facial acne, and hidradenitis suppurativa (HS). It only affects the skin and represents cutaneous characteristics of a spectrum of autoinflammation. Lack of pyogenic sterile arthritis (PA) distinguishes the pyoderma gangrenosum, acne, and hidradenitis suppurativa (PASH) syndrome from pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa (PA-PASH), pyoderma gangrenosum, acne, hidradenitis suppurtiva, and ankylosing spondylitis (PASS), and pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndromes. The exact etiology and pathogenesis of PASH syndrome remain unknown. Both PG and HS are contained in the spectrum of neutrophilic dermatitis, which is considered as an autoinflammatory syndrome. From a pathophysiological point of view, they show similar mechanisms, including neutrophil-rich cutaneous infiltration and overexpression of the interleukin-1 (IL-1) family. These findings provide guidance for these intractable diseases. In this review, we described a case of PASH syndrome in a patient who initially failed to respond to immunosuppressive treatment but responded to a combination of colchicine and thalidomide. We reviewed the relevant literature that focuses on PASH syndrome management.
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