Oral Pemphigus in Children and Adolescents: A Narrative Review of Published Case Reports

Abstract

Background: Pemphigus is a rare group of autoimmune blistering diseases characterized by autoantibody-mediated loss of keratinocyte adhesion, resulting in intraepithelial blister formation involving the skin and mucous membranes. Pemphigus Vulgaris (PV) is the most common type of pemphigus, which usually presents in adulthood and has a prevalence rate of about 2.83 cases per million person years worldwide. The prevalence rate in children and adolescents is relatively uncommon; however, it accounts for about 1.4 to 3.7 percent of all cases of pemphigus vulgaris reported worldwide. The involvement of oral cavity is clinically significant since it can present prior to the disease or as its predominant feature. Hence, this narrative review seeks to review literature on pemphigus with involvement of oral cavity in children and adolescents. Objective: The objective of this study was to review the current literature regarding epidemiology, pathogenesis, clinical presentation, diagnosis, histopathological characteristics, treatment, and outcomes of pemphigus with oral involvement in children and adolescents. Materials and Methods: A literature search was conducted using the PubMed/MEDLINE, Scopus, and Cochrane Library databases to identify relevant studies published between 2015 and 2026. The search strategy included terms related to pemphigus, pediatric patients, and oral manifestations. Articles involving patients younger than 18 years of age with oral involvement were screened according to predefined inclusion criteria. Following database screening, duplicate removal, and full-text assessment, 25 studies comprising a total of 51 patients with documented oral or orofacial involvement were included in the final review. Results: Pemphigus vulgaris was the predominant subtype, with oral lesions representing the initial or sole manifestation in the majority of patients. The gingiva, mucosa, tongue, lip, and palate were the most common affected areas in the mouth. The lesions in these areas usually appeared as painful erosion, ulcers, and desquamative gingivitis. Histopathology with the presence of acantholysis in the suprabasal area and direct immunofluorescence was the most definitive test for diagnosis. Systemic corticosteroids were the mainstay of treatment in conjunction with steroid sparing agents in some cases, with good results in difficult cases using rituximab. Overall, most patients achieved partial or complete clinical remission following appropriate treatment, although relapses were occasionally reported. Conclusions: Pemphigus in children and adolescents is rarely encountered; however, it should be included as a differential diagnosis of erosive/ulcerative lesions. Early identification, diagnosis, and treatment through a collaborative approach from dental practitioners are critical. Further research is required at multiple centers to gain a better understanding of the disease and to develop evidence-based guidelines for diagnosis and treatment of pediatric patients.