Juvenile pemphigus vulgaris: A narrative review

Abstract

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.

Keywords: autoimmune; histopathology; juvenile pemphigus vulgaris; pemphigus; vesiculobullous disease.

Figure 1.

PRISMA flowchart. PRISMA = Preferred Reporting Items for Systematic Literature Reviews and Meta-Analyses.