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Abstract：
Generalized pustular psoriasis (GPP) is a rare chronic inflammatory pustular dermatosis that presents as painful erythema with sterile pustules on nonacral skin. No unified standard and guideline for the treatment of GPP has been established. Several biologics have been tried for GPP, with varying success. Acrodermatitis continua of Hallopeau (ACH) is a very rare disabling variant of pustular psoriasis characterized by sterile pustules on the fingers and toes, including the nail bed. Comparatively, treating ACH is highly challenging due to its commonly therapy-resistant disease course. The pathogenic role of IL-36 signaling axis has been currently identified in GPP development. Spesolimab, the first anti-interleukin-36 receptor biologic, has been approved for treating GPP flares and shown promising results. In view of a shared pathogenesis between GPP and ACH, specolimab may be an effective treatment for ACH. Currently, there is no case and clinical trial data exist on this condition. Therefore, this case was aim to describe real-world experience of spesolimab use in ACH coexisting with GPP. We report an Asian patient with a 16-year-history of GPP and ACH with marked pustulosis on the nail bed and onychodystrophy. He received conventional systemic regimen acitretin, cyclosporine and biologics adalimumab and secukinumab, but experienced relapse for skin lesions and refractory for nail lesions. He was then treated with a single dose of spesolimab in combination with secukinumab, which resulted in skin clearance and nearly complete resolution of nail lesions over a 32-week period. Our observation suggests that spesolimab should be considered for the treatment of ACH, especially in the patients with intractable nail lesions and concomitant GPP.
摘要：
广泛性脓疱型银屑病（3GPP）是一种罕见的慢性炎症性脓疱性皮肤病，表现为疼痛性红斑，在非肢端皮肤上伴有无菌脓疱。目前尚无统一标准和指南来指导如何治疗这一疾病。已经尝试了几种生物制剂，不同的成功。Hallopeau的肢端皮炎连续性（ACH）是脓疱型银屑病的一种非常罕见的致残变体，其特征是手指和脚趾上的无菌脓疱，包括甲床。相对而言,由于ACH通常具有耐药性，因此治疗ACH具有很大的挑战性。IL-36信号传导轴的致病作用目前已被鉴定在3GPP开发中。Spesolimab,第一个抗白细胞介素36受体生物制剂，已被批准用于治疗3GPP耀斑，并显示出有希望的结果。鉴于3GPP和ACH之间的共同发病机制，specolimab可能是ACH的有效治疗方法。目前,这种情况没有病例和临床试验数据。因此,该病例旨在描述Spesolimab在ACH中的实际使用经验,并与3GPP共存.我们报告了一名亚洲患者，有16年的3GPP和ACH病史，甲床上有明显的脓疱病和甲营养不良。他接受了阿曲汀的常规全身治疗方案，环孢菌素和生物制剂阿达木单抗和苏金单抗，但皮肤病变复发，指甲病变难治。然后他接受了单剂量的spesolimab联合苏金单抗治疗，在32周的时间内，皮肤清除和指甲病变几乎完全消退。我们的观察表明，spesolimab应考虑用于治疗ACH，尤其是在患有顽固性指甲病变和伴发3GPP的患者中。
