The Ohio State University College of Medicine, Columbus, OH, 43210, USA.
Department of Dermatology, The Ohio State University Wexner Medical Center, Columbus, OH, 43210, USA.
Arch Dermatol Res. 2024 Sep 11;316(8):610. doi: 10.1007/s00403-024-03356-8.
Darier disease and Hailey-Hailey disease are rare autosomal dominant genodermatoses that negatively impact patient quality of life. In addition, they pose challenges to dermatologists who manage these diseases. There is currently no treatment that reliably induces remission for either disease, leaving patients dependent on symptom management. Oral and topical retinoids are the most commonly used therapies but have numerous side effects that often lead to discontinuation or inability to tolerate long-term treatment (Burge and Wilkinson in J Am Acad Dermatol 27:40-50, 1992). Due to the rarity of these diseases, there are no clinical trials investigating treatment options for the persistent flares patients experience. In light of this, dermatologists have tried various methods used in the management of other inflammatory disorders including photodynamic therapy (PDT). A systematic review was conducted to investigate this treatment option which yielded a total of 12 studies that had reported the use of photodynamic therapy (PDT) as a treatment option for Darier or Hailey-Hailey disease. Though results showed that PDT can induce disease remission for up to several months or years, there are many unanswered questions that need to be addressed before adopting PDT as a leading treatment option for those genodermatoses. In particular, cost, tolerability, and efficacy and safety in patients who are skin of color need to be further studied. Lastly, recommendations on treatment duration, number of sessions, photosensitizing agents, lasers, and continuation or discontinuation of topical and systemic medications need to be appraised before formal recommendations can be made.
Darier 病和 Hailey-Hailey 病是罕见的常染色体显性遗传皮肤病，会严重影响患者的生活质量。此外，它们也给皮肤科医生的治疗带来了挑战。目前，这两种疾病都没有可靠的治疗方法可以诱导缓解，患者只能依赖症状管理。口服和局部维 A 酸类药物是最常用的治疗方法，但有许多副作用，常导致治疗中断或无法耐受长期治疗（Burge 和 Wilkinson in J Am Acad Dermatol 27:40-50, 1992）。由于这些疾病较为罕见，因此没有临床试验来研究针对患者持续发作的治疗选择。鉴于此，皮肤科医生尝试了各种用于治疗其他炎症性疾病的方法，包括光动力疗法（PDT）。进行了一项系统评价来研究这种治疗选择，共纳入了 12 项报告 PDT 作为 Darier 病或 Hailey-Hailey 病治疗选择的研究。尽管结果表明 PDT 可以诱导疾病缓解长达数月或数年，但在将 PDT 作为这些遗传性皮肤病的主要治疗选择之前，仍有许多悬而未决的问题需要解决。特别是，需要进一步研究 PDT 的成本、耐受性、在有色人种患者中的疗效和安全性。最后，在提出正式建议之前，需要评估治疗持续时间、治疗次数、光敏剂、激光以及局部和全身药物的继续或停止使用等方面的建议。
