A rare case of progressive neonatal pemphigus vulgaris treated successfully with intravenous immunoglobulin

Pemphigus vulgaris is a rare autoimmune blistering disorder of the skin and mucous membranes, particularly during pregnancy. The transplacental transfer of maternal immunoglobulin G autoantibodies against desmoglein-3 to the fetus can result in neonatal pemphigus vulgaris (NPV). Although NPV is typically transient and benign, resolving without specific treatment or long-term sequelae, its impact on both the pregnancy and the fetus underscores the importance of early recognition. Prompt diagnosis is crucial to differentiate NPV from other blistering conditions, such as infections, epidermolysis bullosa, incontinentia pigmenti, and porphyrias, which require specific interventions. We present a case of a male neonate who developed blisters on the first day of life, which later involved mucosal surfaces, and responded well to treatment with intravenous immunoglobulin.