Pemphigus vegetans: insights from a systematic review of the literature

Pemphigus vegetans (PVg) is a rare variant of pemphigus vulgaris with distinct clinical and histological features, with fewer than 200 cases of patients with the condition reported.

To systematically review cases of patients with PVg and summarize clinical, histopathological, immunological features, treatment and outcomes.

Following PRISMA guidelines, PubMed, Embase and Web of Science were searched (1889 to January 2025) using 'pemphigus AND vegetans'. Eligible studies included case reports, series and observational studies. Data on demographics, subtypes, histopathology, immunopathology, treatment and outcomes were extracted.

In total, 197 patients with the condition from 148 studies were identified. The mean age was 52.2 (SD 20.2) years with near-equal sex distribution. Oral mucosa was the commonest initial site (44.7%, 73/163); flexural areas such as the groin and axilla were frequently involved. Reported triggers included drugs, infections and vaccination. In 89 patients where clinical subtypes were reported, the Hallopeau variant was found in 47 (53%). When treatment outcomes were reported by subtype, patients with the Hallopeau variant demonstrated higher sustained remission rates (68%, 28/41) and lower mortality (2%, 1/41) compared with those with the Neumann variant [52% (16/31) sustained remission; 16% (5/31) mortality]. Key histological findings included suprabasal acantholysis (84.9%, 158/186) and eosinophilic microabscesses (65.1%, 121/186). Direct immunofluorescence showed intercellular IgG (92.4%, 121/131) and C3 (53.4%, 70/131). Oral corticosteroids (89.5%, 145/162) were the most used therapy. Relapse occurred in 23.0% (35/152) of patients achieving remission.

This review highlights the need for standardized diagnostic criteria, improved immunological profiling and collaborative data collection to guide evidence-based management.