Pediatric Bullous Pemphigoid: Clinical Spectrum, Diagnosis, Management, and Future Perspectives—A Narrative Review

Background/Objectives: Bullous pemphigoid (BP) in children is a rare autoimmune subepidermal blistering condition that shows significant clinical variability depending on age. This narrative review summarizes the available literature on the epidemiology, etiology, clinical manifestations, diagnosis, management, outcomes, and future perspectives of pediatric BP. Methods: A search of the PubMed/MEDLINE and Scopus databases from their inception to July 2026 was conducted for English-language articles on BP in patients younger than 18 years old. After record screening and full-text assessment, 55 publications were selected and narratively synthesized. Results: Infants with BP frequently present with acral and palmoplantar blistering, whereas older children and adolescents show more heterogeneous clinical manifestations. BP180 is the principal autoantigen, although antigenic heterogeneity has been reported. Diagnosis requires integration of clinical, histopathological, and immunopathological findings. Linear IgG and/or C3 deposition along the basement membrane zone on direct immunofluorescence (DIF) is an important but non-specific finding. Salt-split skin testing and antigen-specific serology may assist in distinguishing BP from other autoimmune subepidermal blistering diseases. Corticosteroids are the most frequently reported initial treatment, while dapsone and other steroid-sparing agents have been used in selected patients. Intravenous immunoglobulin (IVIG), immunosuppressants, and targeted therapies have primarily been reported in refractory cases, with evidence derived predominantly from case reports and small series. Conclusions: The prognosis reported in the pediatric literature is generally favorable, although there are some reports of resistant or recurrent cases. However, relapse rates in the long-term are not known. There is a need for multicenter prospective trials and standardization of outcomes.