Stege Henner, Haist Maximilian, Schultheis Michael, Pawlowski Johannes, Wittmann Miriam, Grabbe Stephan, Butsch Florian
Department of Dermatology, University Medical Center Mainz, 55131 Mainz, Germany.
J Clin Med. 2024 Aug 22;13(16):4947. doi: 10.3390/jcm13164947.
Lichen planopilaris (LPP) is an inflammatory cicatricial alopecia characterized by an irreversible destruction of the hair follicle resulting in its permeant destruction. The clinical presentation of LPP is a progressive patchy scarring alopecia. A variety of systemic agents is used to treat LPP with varying success. The aim of this retrospective, real-life analysis was to evaluate the treatment of hydroxychloroquine for LPP. In this retrospective, single-center study, we analyzed 110 patients with LPP and frontal fibrosing alopecia (FFA) who received treatment over a 12-month period from March 2014 to March 2021 at the Department of Dermatology, University of Mainz Medical Center. Patient records were analyzed for response to treatment, co-morbidities, disease progression-free survival (DPFS), and safety. Clinical parameters associated with treatment response were determined with Cox regression modelling and logistic regression. Overall, 77 of 110 patients were treated with a systemic agent. There was a clear association between LPP and the occurrence of Hashimoto thyroiditis. Topical treatment with corticosteroids did not improve clinical symptoms in the majority of patients (15 out of 101). In 71% of patients treated with systemic cyclosporine A and 62% of patients treated with hydroxychloroquine, we observed a significant resolution of the inflammatory process, which correlated with a robust durable clinical response ( < 0.001). Toxicity was observed in 17% ( = 9) of patients receiving systemic treatment with hydroxychloroquine and correlated with the duration of systemic treatment ( < 0.001). Treatment discontinuation was associated with a flare-up of clinical symptoms (29%), which required the re-initiation of second-line therapy in 13 out of 51 patients. Overall, the initiation of second-line treatment, either hydroxychloroquine or Cyclosporine A (CsA), yielded positive results, especially in the patient cohort treated with hydroxychloroquine (overall response rate, ORR = 100%), who showed disease progression during CsA or retinoids. : Our results from this contemporary cohort of patients with LPP and FFA indicate that hydroxychloroquine and cyclosporine are effective systemic agents in decreasing clinical symptoms. However, our data also show that the discontinuation of treatment is often associated with the exacerbation of clinical symptoms. Response rates to second-line treatment were especially favorable in the patient cohort with hydroxychloroquine.
扁平苔藓性毛发角化病（LPP）是一种炎症性瘢痕性脱发，其特征是毛囊发生不可逆破坏，导致永久性损毁。LPP的临床表现为进行性斑片状瘢痕性脱发。多种全身性药物用于治疗LPP，但疗效各异。这项回顾性真实病例分析的目的是评估羟氯喹治疗LPP的效果。在这项回顾性单中心研究中，我们分析了2014年3月至2021年3月期间在美因茨大学医学中心皮肤科接受治疗的110例LPP和额部纤维性脱发（FFA）患者。分析患者记录，以了解治疗反应、合并症、无疾病进展生存期（DPFS）和安全性。通过Cox回归模型和逻辑回归确定与治疗反应相关的临床参数。总体而言，110例患者中有77例接受了全身性药物治疗。LPP与桥本甲状腺炎的发生之间存在明显关联。大多数患者（101例中的15例）使用皮质类固醇进行局部治疗后临床症状并未改善。在接受全身性环孢素A治疗的患者中，71%以及接受羟氯喹治疗的患者中，62%观察到炎症过程有显著消退，这与持久的临床反应相关（P<0.001）。在接受羟氯喹全身性治疗的患者中，17%（n = 9）出现毒性反应，且与全身性治疗的持续时间相关（P<0.001）。治疗中断与临床症状复发相关（29%），51例患者中有13例因此需要重新开始二线治疗。总体而言，开始使用二线治疗药物，即羟氯喹或环孢素A（CsA），均取得了积极效果，尤其是在接受羟氯喹治疗的患者队列中（总缓解率，ORR = 100%），这些患者在使用CsA或维甲酸类药物期间出现了疾病进展。结论：我们对这个LPP和FFA患者当代队列的研究结果表明，羟氯喹和环孢素是减轻临床症状的有效全身性药物。然而，我们的数据也表明，治疗中断往往与临床症状加重相关。二线治疗的缓解率在接受羟氯喹治疗的患者队列中尤其良好。
