Pemphigus vulgaris (PV) is an autoimmune bullous disease. It has a very low incidence in childhood with very few cases reported in literatures. A 6-year-old female child had non-healing oral erosions for a month and recent appearance of genital erosions. Differential diagnoses considered were oral candidiasis, herpetic stomatitis, and PV. On investigating further, KOH mount showed Candidial hyphae and spores. Serology for herpes viruses was negative. Tzanck smear showed acantholytic cells. Obtaining biopsy from oral mucosa was difficult; hence, indirect immunofluorescence was done which showed significant titers of anti-epidermal (pemphigus) antibodies. Anti-desmoglein 1 and 3 antibodies measured by ELISA were also positive. Final diagnosis was PV. The patient was started on oral prednisolone. However, when relapsed, considering the long-term adverse effects of systemic corticosteroids on growth of the child, two infusions of rituximab, 15 days apart, were administered after clearance from a pediatrician and appropriate laboratory work up. Outcome: There was complete remission after 1 month of rituximab. Prednisolone was tapered off quickly and withdrawn completely. There was a persistent decline in anti-desmoglein antibodies during follow-up. No severe adverse effects were documented. The child remained in remission up to 12 months of follow-up. Discussion: This is a very rare case of childhood PV. Rituximab appears to be an effective therapeutic option in children with pemphigus. Further studies will help in defining protocols for use of rituximab in children.
Read full abstract