Ständer Sascha, Syring Felicia, Ludwig Ralf J, Thaçi Diamant
Department of Dermatology, University of Lübeck, Lübeck, Germany.
Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany.
Front Med (Lausanne). 2020 Oct 15;7:543944. doi: 10.3389/fmed.2020.543944. eCollection 2020.
Palmoplantar pustular psoriasis (PPPP) is a debilitating inflammatory skin disorder of the palms and soles that poses a high burden on affected patients. Satisfactory treatment response is rarely achieved using current treatment options, little is known about the potential benefit of the PDE4 inhibitor apremilast in the treatment of refractory PPPP patients. We aimed to evaluate the use of apremilast in PPPP patients. Six patients, four with severe physician global assessment (PGA) = 3 on a scale of 0-4 and two with very severe (PGA = 4) treatment-refractory PPPP [mean age (years ± SD): 56.2 ± 15.6], were included in this study. Five patients had concomitant psoriatic arthritis (PsA). Prior to apremilast administration, topical corticosteroids, psoralen-UVA and multiple systemic oral and biologic anti-inflammatory treatments were insufficient to improve their skin condition or had to be discontinued due to adverse events. Apremilast (titrated to a maintenance dose of 30 mg 2x/d) was commenced in all patients with clinical follow-up over 18 months. Within the first 4 weeks of treatment, each patient's symptoms improved as assessed by PGA score. At 3 months, four patients had a mild PGA score and two were cleared from PPPP. After 18 months of follow-up, three patients improved from PGA = 3 to PGA = 1 and one patient from PGA = 4 to PGA = 1. Two patients discontinued treatment, one due to a lack of efficacy against PsA and the other to a desire to have a child. However, both patients recorded improvements before discontinuing treatment. Apremilast may be a promising treatment option for refractory and severely affected PPPP patients. Our observation, however, requires further validation.
掌跖脓疱病(PPPP)是一种累及手掌和足底的使人衰弱的炎症性皮肤病,给患者带来沉重负担。使用目前的治疗方案很少能获得令人满意的治疗反应,关于磷酸二酯酶4(PDE4)抑制剂阿普司特在治疗难治性PPPP患者中的潜在益处知之甚少。我们旨在评估阿普司特在PPPP患者中的应用。本研究纳入了6例患者,其中4例医生整体评估(PGA)为重度(0 - 4级中为3级),2例为极重度(PGA = 4级)的治疗难治性PPPP患者[平均年龄(岁±标准差):56.2±15.6]。5例患者合并银屑病关节炎(PsA)。在给予阿普司特之前,外用糖皮质激素、补骨脂素 - 紫外线A以及多种全身性口服和生物抗炎治疗均不足以改善他们的皮肤状况,或因不良事件而不得不停药。所有患者均开始使用阿普司特(滴定至维持剂量30 mg,每日2次),并进行了超过18个月的临床随访。在治疗的前4周内,根据PGA评分评估,每位患者的症状均有改善。3个月时,4例患者PGA评分为轻度,2例PPPP症状消退。随访18个月后,3例患者从PGA = 3改善至PGA = 1,1例患者从PGA = 4改善至PGA = 1。2例患者停止治疗,1例是因为对PsA治疗无效,另1例是因为想要孩子。然而,两名患者在停药前病情均有改善。阿普司特可能是难治性和重度PPPP患者的一种有前景的治疗选择。然而,我们的观察结果需要进一步验证。