Palmieri Claudia, Noviello Chiara, Moscara Lorenza, Stefanizzi Pasquale, Berti Irene, Tafuri Silvio, Arbo Anna
Interdisciplinary Department of Medicine, Aldo Moro University of Bari, Bari, Italy.
Burlo Garofalo Scientific Institute for Research, Hospitalization and Healthcare, Trieste, Italy.
Hum Vaccin Immunother. 2025 Dec;21(1):2494457. doi: 10.1080/21645515.2025.2494457. Epub 2025 Apr 20.
Epidermolysis bullosa is a rare genetic disorder characterized by skin fragility and blistering, resulting from mutations in dermal-epidermal junction structural proteins. Disease severity varies, with some cases involving extensive skin damage and complications secondary to chronic inflammation. Patients with immune-mediated dermatological conditions face a heightened risk of Herpes Zoster and Post-Herpetic Neuralgia, particularly during immunosuppressive treatments. A 10-year-old female with Recessive Dystrophic Epidermolysis Bullosa received off-label Recombinant adjuvanted Zoster Vaccine (RZV) - approved in individuals 18 years of age and older - before initiating Janus kinase inhibitor therapy, required to manage the underlying condition. Two standard RZV doses were administered on February 22 and April 29, 2024. No immediate vital sign alterations nor Adverse Events Following Immunization were documented in the first 7 days after immunization. A 6-month follow-up revealed no relevant emergent clinical events nor Herpes Zoster episodes. In conclusion, the case offers preliminary insights into RZV safety for high-risk pediatric patients. However, robust safety and efficacy studies are warranted to support the implementation of RZV for vulnerable individuals under 18 years of age.
大疱性表皮松解症是一种罕见的遗传性疾病,其特征为皮肤脆弱和水疱形成,由真皮-表皮连接结构蛋白的突变引起。疾病严重程度各不相同,一些病例会出现广泛的皮肤损伤以及慢性炎症继发的并发症。患有免疫介导皮肤病的患者患带状疱疹和带状疱疹后神经痛的风险增加,尤其是在免疫抑制治疗期间。一名患有隐性营养不良型大疱性表皮松解症的10岁女性,在开始使用用于治疗基础疾病的 Janus 激酶抑制剂治疗之前,接受了未获批准的重组佐剂带状疱疹疫苗(RZV,已批准用于18岁及以上人群)。于2024年2月22日和4月29日接种了两剂标准剂量的RZV。免疫接种后的前7天内未记录到生命体征的即刻改变或免疫接种后不良事件。6个月的随访显示没有相关的紧急临床事件或带状疱疹发作。总之,该病例为RZV在高危儿科患者中的安全性提供了初步见解。然而,需要进行有力的安全性和有效性研究,以支持在18岁以下的易感个体中使用RZV。