De Wet J, Jordaan H F, Kannenberg S M, Tod B, Glanzmann B, Visser W I
Division of Dermatology, Department of Medicine, University of Stellenbosch and Tygerberg Academic Hospital, Tygerberg, Cape Town, South Africa.
Dermatol Online J. 2017 Dec 15;23(12):13030/qt82d4m2zw.
PASH syndrome (pyoderma gangrenosum, acne, and suppurative hidradenitis) forms part of the spectrum of autoinflammatory diseases. We report an unusual case of PASH syndrome in a patient with end-stagerenal disease (ESRD) who was successfully treated with the tumor necrosis factor inhibitor, adalimumab. The case underscores the challenges associatedwith the treatment of PASH syndrome as well as the ongoing search to establish a genetic basis for the syndrome. Renal impairment has been reported in association with pyoderma gangrenosum but has notbeen described in PASH syndrome. We believe this to be the first reported case of a patient who developed PASH syndrome in the setting of ESRD.
PASH综合征（坏疽性脓皮病、痤疮和化脓性汗腺炎）是自身炎症性疾病谱的一部分。我们报告了一例终末期肾病（ESRD）患者发生PASH综合征的罕见病例，该患者接受肿瘤坏死因子抑制剂阿达木单抗治疗成功。该病例凸显了PASH综合征治疗相关的挑战以及对该综合征遗传基础的持续探索。已有坏疽性脓皮病伴发肾功能损害的报道，但PASH综合征中尚未见描述。我们认为这是首例在ESRD背景下发生PASH综合征的报道病例。
