Intravenous immunoglobulin as an effective treatment for refractory pemphigus vegetans: A case report

Pemphigus vegetans (PVeg), which affects < 2% of pemphigus cases, presents a therapeutic challenge when refractory to conventional treatments. Herein, we describe the case of a 58-year-old woman diagnosed with Hallopeau-type PVeg who demonstrated a complete response to intravenous immunoglobulin (IVIG) after failing multiple conventional therapies. The patient exhibited vegetative plaques at multiple sites, with notable nail involvement. Her disease remained poorly controlled despite a 7-year course of systemic corticosteroids, immunosuppressants, and rituximab. Two courses of IVIG (5% concentration, 2 g/kg) resulted in the complete resolution of the disease. This case highlights IVIG’s therapeutic potential in refractory PVeg and emphasizes the importance of recognizing atypical manifestations, including nail involvement. While this single case yielded promising results, future studies must validate the role of IVIG in the management of treatment-resistant PVeg.