A Rare Case of Juvenile Pemphigus Vulgaris Treated with Intravenous Immunoglobulin / 대한피부과학회지

ABSTRACT

Juvenile pemphigus vulgaris (JPV) is a rare variant of pemphigus vulgaris (PV) occurring in childhood and adolescence that has similar symptoms and the same histological and immunopathological features as classic adult PV. Although rare, advanced cases of JPV can be fatal due to secondary sepsis. Many patients with JPV are misdiagnosed and therefore not properly treated in the early stages of the disease. Although systemic corticosteroids are the therapeutic mainstay, long-term corticosteroid use has various adverse effects. Intravenous immunoglobulin (IVIG) was recently reported to reduce the side effects of corticosteroids. Here, we report a case of JPV in a 14-year-old boy treated with IVIG.