Rituximab in Childhood and Juvenile Pemphigus Vulgaris: A Systematic Review.

Pemphigus vulgaris (PV) is a chronic autoimmune blistering disorder characterized by the loss of intraepithelial adhesion, affecting the skin and mucous membranes. Both males and females are affected, although it predominantly affects females in their fifth and sixth decades of life. Approximately 1.4 to 3.7% of PV cases occur in the pediatric population (≤18 years of age), and may be classified into childhood/pediatric PV, which affects individuals under 12 years old, and juvenile/adolescent PV, affecting those between 12 and 18 years old. Due to its rare occurrence in children and adolescents, there is often a delay in diagnosis and treatment in this age group. A systematic literature search was conducted on MEDLINE/PubMed, Web of Science, EMBASE, SCOPUS, and Cochrane Library databases to evaluate the efficacy of rituximab (RTX) in childhood and juvenile PV patients. The Joanna Briggs Institute (JBI) Critical Appraisal Checklist was employed to assess the risk of bias in case reports and series, while the Cochrane ROBINS-I tool was utilized for evaluating observational studies or non-randomized intervention studies. A total of 18 studies encompassing 46 juvenile or childhood PV patients in the pediatric and adolescent age groups were included for qualitative synthesis. The studies included nine case reports, two case series, five retrospective studies, one prospective study, and one open-label pilot study. Almost all cases of childhood and juvenile PV achieved either complete or partial remission after undergoing RTX treatment during the final follow-up periods. Furthermore, most cases reported no relapse, and only minor adverse events were noted in the RTX treatment group. Despite its potential benefits, the utilization of RTX in pediatric patients raises concerns due to the scarcity of evidence and the absence of controlled studies specific to this age group. Further exploration is necessary to establish a standardized treatment regimen for RTX in pediatric PV, which involves identifying the optimal dosage, frequency, treatment cycle duration, and maintenance therapy duration.

Eye and lid involvement as an uncommon feature of pemphigus foliaceus in a pediatric patient.

Pemphigus foliaceus (PF) is an autoimmune blistering disorder which affects the superficial layers of the epidermis with rare mucosal involvement. We present the case of a 12-year-old girl with PF involving the eyes and eyelids. A literature review of pediatric nonendemic PF revealed another two cases with ocular manifestations. Eyelid involvement is an uncommon feature of PF that should be properly identified and treated.

Management of pediatric pemphigus vulgaris with rituximab: A case report and review of the literature.

Pemphigus vulgaris (PV), an acquired autoimmune bullous disease, is caused by autoantibodies targeting desmosomal proteins in the skin and mucous membranes. Recent data from the adult PV population supports the use of rituximab, a chimeric anti-CD20 IgG1 antibody, as a primary treatment strategy, but limited data exist regarding treatment in the pediatric population. We report the case of a 13-year-old male with PV treated successfully with systemic corticosteroids and rituximab, and review the literature supporting the treatment of pediatric PV with rituximab.

Pemphigus herpetiformis in a 4-year-old child: Case report and review of the literature.

Pemphigus herpetiformis (PH) is a rare form of pemphigus, especially when occurring in childhood. Misdiagnosis is common in this age group. The disease exhibits diverse clinical and histological aspects. Further immunological investigations should be performed in order to make the right diagnosis with a correct management strategy.

Rituximab therapy in pediatric pemphigus patients: A retrospective analysis of five Turkish patients and review of the literature.

BACKGROUND/OBJECTIVES: There is inadequate knowledge regarding rituximab (RTX) administration in autoimmune bullous diseases (AIBDs), disease prevalence, clinical characteristics, and treatment outcomes within pediatric populations due to the rarity of AIBDs affecting the pediatric age group. The aim of this retrospective analysis was to evaluate the effectiveness, safety of RTX, and treatment outcomes in Turkish pediatric patients with pemphigus vulgaris (PV) and to review the literature. METHODS: Five patients under 18 years of age and diagnosed with PV received RTX treatment and were identified in four dermatology departments of Turkey. RESULTS: The mean age of the patients at the time of RTX therapy initiation was 15 years (range: 11-17 years), and the total duration of follow-up after RTX therapy was 42.6 months (range: 19-60 months). All patients showed a clinical response. At the last visit, complete remission off therapy was achieved in three patients. The remaining two patients achieved partial remission off therapy. No adverse events were observed. CONCLUSIONS: This retrospective case series of five pediatric patients showed that RTX treatment can be effective and safe for the treatment of recalcitrant PV in pediatric patients. With increasing evidence, RTX is a good treatment choice in adults and pediatric patients with pemphigus.