Debeuf Marie-Eline P H, Knoops Kèvin, López-Iglesias Carmen, Lookermans Eline, Nelemans Patty J, Abdul Hamid Myrurgia, van Geel Michel, Gostynski Antoni H, Steijlen Peter M, Bolling Marieke C, Verstraeten Valerie L R M
Department of Dermatology, Centre of Expertise for Genodermatoses, Maastricht University Medical Centre+, Maastricht, The Netherlands.
GROW Research Institute for Oncology and Developmental Biology, Maastricht University, Maastricht, The Netherlands.
J Eur Acad Dermatol Venereol. 2025 May;39(5):1038-1045. doi: 10.1111/jdv.20351. Epub 2024 Oct 1.
Hailey-Hailey disease (HHD) is a rare genetic therapy-resistant blistering disease with great disease burden. Treatment is currently focused on symptomatic relief. Er:YAG ablative laser therapy is a therapeutic modality with promising results, though evidence is currently scarce.
To analyse the effect of Er:YAG ablative laser therapy on clinical remission of erosive plaques in HHD and on patient's quality of life (QoL).
In this observational study eight patients were included and 77 erosive plaques were treated once only by Er:YAG laser and assessed for clinical remission. QoL was evaluated by obtaining Skindex-29 and DLQI questionnaires before laser therapy, 6 weeks and 3 years after laser therapy. Skin biopsies were taken to evaluate the depth of laser ablation. The intercellular distance between keratinocytes, the number of desmosomes and intermediate filament distribution were studied by electron microscopy before and after laser therapy and in clinically uninvolved skin and were compared to subjects without HHD.
One single Er:YAG laser ablation to mid-dermis resulted in complete remission of 97.4% of HHD plaques (75/77) after median 38 months (range 7-63 months) and significantly improved QoL. Laser therapy restored the number of desmosomes, decreased intercellular distance and diminished perinuclear retraction of keratin filaments to a level comparable to the patient's clinically uninvolved skin. After laser ablation, the skin showed significantly fewer ultrastructural aberrations compared to the patient's clinically uninvolved skin and rather resembled the skin of healthy control individuals.
One single Er:YAG laser treatment resulted in long-term remission of HHD and significantly improved QoL. Our findings support a greater role for ablative laser surgery in the management of this recalcitrant disorder.
黑利-黑利病（HHD）是一种罕见的、对基因治疗耐药的水疱性疾病，疾病负担较重。目前的治疗主要集中在症状缓解。铒：钇铝石榴石（Er:YAG）剥脱性激光治疗是一种有前景的治疗方式，不过目前证据较少。
分析Er:YAG剥脱性激光治疗对HHD糜烂性斑块临床缓解及患者生活质量（QoL）的影响。
在这项观察性研究中，纳入了8例患者，77个糜烂性斑块仅接受一次Er:YAG激光治疗，并评估其临床缓解情况。通过在激光治疗前、治疗后6周和3年获取Skindex-29和皮肤病生活质量指数（DLQI）问卷来评估生活质量。进行皮肤活检以评估激光消融的深度。在激光治疗前后以及临床未受累皮肤中，通过电子显微镜研究角质形成细胞之间的细胞间距离、桥粒数量和中间丝分布，并与无HHD的受试者进行比较。
单次Er:YAG激光消融至真皮中层，在中位38个月（范围7 - 63个月）后，97.4%的HHD斑块（75/77）完全缓解，且生活质量显著改善。激光治疗恢复了桥粒数量，减小了细胞间距离，并使角蛋白丝的核周退缩减少至与患者临床未受累皮肤相当的水平。激光消融后，与患者临床未受累皮肤相比，皮肤超微结构异常明显减少，更类似于健康对照个体的皮肤。
单次Er:YAG激光治疗导致HHD长期缓解，并显著改善生活质量。我们的研究结果支持剥脱性激光手术在这种难治性疾病管理中发挥更大作用。
