Diagnosis, Treatment, and Long-Term Outcomes of Pediatric Pemphigus: A Retrospective Study at Tertiary Medical Centers

This retrospective study of 12 pediatric patients with pemphigus at tertiary medical centers in Israel highlights the diagnostic challenges and treatment outcomes of this rare autoimmune blistering disease. The mean age at diagnosis was 10.7 years, with an equal male-to-female ratio, and diagnosis was often delayed by up to 36 months. Initial treatment with systemic corticosteroids led to complete remission in only 25% of patients, while rituximab, used in 41.7% of cases, showed promise as a second or third-line treatment. Dapsone was the most common steroid-sparing agent, though less effective than in adults. Long-term follow-up revealed ongoing active disease in some patients and noted comorbidities such as ADHD. The study suggests that outpatient treatment may be as effective as hospitalization for high-dose corticosteroids and emphasizes the need for further research to improve management and long-term outcomes.