Pemphigus vulgaris (PV) is an autoimmune disorder involving the mucocutaneous tissues. Pathogenesis of this disease causes auto-antibodies against desmogleins in desmosomes which leads to intraepithelial blister formation. It is a rare but potentially life-threatening disease with a prevalence of 1 to 9 per 1 x 109. The disease has an equal sex predilection and commonly occurs in the 5th and 6th decade of life. Clinically, oral lesions are more evident than skin lesions. Diagnosis of the disease is by clinicopathological correlation with the definitive diagnosis requiring immunofluorescent investigations. The mainstay of treatment involves immunosuppression through the use of corticosteroids and other steroid-sparing agents like dapsone, azathioprine, and methotrexate. Presentation of this entity in an adolescent is a rare occurrence, and this may lead to misdiagnosis of the condition. Quality of life of these patients can be improved by controlling the disease through early diagnosis and necessary management. In this article, we report a case of a 15-year-old male patient diagnosed with Juvenile PV (JPV) and discuss the possibility of pemphigusvulgaris as a differential diagnosis for chronic generalized oral ulceration in an adolescent with a brief review of the literature.
