Wen Pengfei, Liu Chuan, Wang Tingting, Jiang Xian, Wang Ping, Wang Sheng
Department of Dermatovenerology, West China Hospital, Sichuan University, Chengdu, China.
The Department of Dermatology, First Affiliated Hospital of Chongqing Medical University, Chongqing, China.
Front Immunol. 2024 Feb 23;15:1338285. doi: 10.3389/fimmu.2024.1338285. eCollection 2024.
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.
泛发性脓疱型银屑病（GPP）是一种罕见的慢性炎症性脓疱性皮肤病，表现为非肢端皮肤出现疼痛性红斑并伴有无菌性脓疱。目前尚未建立GPP治疗的统一标准和指南。已对多种生物制剂用于GPP进行了尝试，疗效各异。Hallopeau连续性肢端皮炎（ACH）是脓疱型银屑病一种非常罕见的致残性变异型，其特征为手指和脚趾（包括甲床）出现无菌性脓疱。相比之下，由于ACH通常病程对治疗耐药，因此治疗极具挑战性。目前已确定IL-36信号轴在GPP发病机制中的作用。首个抗白细胞介素-36受体生物制剂司库奇尤单抗已获批用于治疗GPP发作，并显示出有前景的结果。鉴于GPP和ACH之间存在共同的发病机制，司库奇尤单抗可能是治疗ACH的有效药物。目前，关于这种情况尚无病例和临床试验数据。因此，本病例旨在描述司库奇尤单抗用于合并GPP的ACH的真实世界经验。我们报告一名有16年GPP和ACH病史的亚洲患者，其甲床有明显脓疱病和甲营养不良。他接受了阿维A、环孢素等传统全身治疗方案以及生物制剂阿达木单抗和司库奇尤单抗，但皮肤病变复发，甲病变难治。然后他接受了单剂量司库奇尤单抗联合司库奇尤单抗治疗，在32周内皮肤病变消退，甲病变几乎完全缓解。我们的观察表明，应考虑使用司库奇尤单抗治疗ACH，尤其是对于有难治性甲病变且合并GPP的患者。
