Sriram Shyamkumar, Jayakanth Mambakkam, Alam Tanveer, Saeed Shazina, Hasan Shamimul
Department of Rehabilitation and Health Services, College of Health and Public Service, University of North Texas, Denton, TX.
Department of Internal Medicine, Patiala Heart Institute, Patiala, India.
Medicine (Baltimore). 2025 May 23;104(21):e42611. doi: 10.1097/MD.0000000000042611.
Pemphigus vulgaris is a chronic autoimmune dermatosis that primarily affects the skin and mucous membranes. Juvenile pemphigus vulgaris (JPV), a pediatric variant of pemphigus vulgaris, displays unusual features that may mimic other mucosal lesions. This similarity leads to diagnostic and therapeutic difficulties, often causing delays in diagnosis. Primary treatment consists of systemic corticosteroids and immunosuppressive agents, with biological therapies, such as rituximab, emerging as potential alternatives. Timely diagnosis and treatment are essential to improve prognosis and reduce complications related to long-term immunosuppressive therapy. A comprehensive electronic and manual literature search was conducted using various databases to consolidate the existing literature on JPV, focusing on its epidemiology, pathogenesis, clinical presentation, diagnosis, treatment options, and long-term outcomes in pediatric patients. A detailed review focusing on JPV has been conducted over the last 10 years (2014-2024). A literature review identified 37 cases of JPV in 16 studies. Owing to the rarity of JPV, much of the available evidence relies on case reports and case series, resulting in poorly defined response rates to different treatment options. Additionally, long-term monitoring of patients with disease recurrence is lacking. By identifying gaps in the current knowledge and offering a comprehensive overview, this review seeks to enhance awareness and guide future research and management strategies for JPV.
寻常型天疱疮是一种主要累及皮肤和黏膜的慢性自身免疫性皮肤病。青少年寻常型天疱疮（JPV）是寻常型天疱疮的一种儿科变体，具有一些可能与其他黏膜病变相似的不寻常特征。这种相似性导致诊断和治疗困难，常常造成诊断延误。主要治疗方法包括全身使用皮质类固醇和免疫抑制剂，生物疗法如利妥昔单抗也逐渐成为潜在的替代方案。及时诊断和治疗对于改善预后以及减少与长期免疫抑制治疗相关的并发症至关重要。通过使用各种数据库进行全面的电子和手动文献检索，以整合关于JPV的现有文献，重点关注其在儿科患者中的流行病学、发病机制、临床表现、诊断、治疗选择和长期预后。在过去10年（2014 - 2024年）对JPV进行了详细的综述。文献综述在16项研究中确定了37例JPV病例。由于JPV罕见，现有证据大多依赖病例报告和病例系列，导致对不同治疗选择的有效率定义不明确。此外，缺乏对疾病复发患者的长期监测。通过识别当前知识中的空白并提供全面概述，本综述旨在提高对JPV的认识，并指导未来对JPV的研究和管理策略。
