A Case of Hallopeau Type Pemphigus Vegetans Successfully Treated with High-dose Intravenous Immunoglobulin(IVIG)Therapy

Abstract

An 83-year-old woman had complained of pain of her genital region for one month. She was suspected of having a malignant tumor at a nearby clinic and was admitted to our hospital for further examination. At her first visit, verrucous vegetation was seen on the entire genital region. After a limited time, erosions at the axilla and lips and thickening of the tongue also appeared. Histologically, acanthosis and a microabscess containing numerous eosinophils were observed at the lesional epidermis. Direct immunofluorescence analysis revealed the deposition of IgG at keratinocyte surfaces. Her serum levels of anti-desmoglein (Dsg) 1 and anti-Dsg3 antibodies were elevated. Based on clinical, histological, and laboratory findings, this case was diagnosed as Hallopeau type pemphigus vegetans. Although she developed a widespread erosion that was not well controlled with oral corticosteroids only, the erosion was controlled by combining corticosteroids with high-dose intravenous immunoglobulin therapy.