Pemphigus vegetans: insights from a systematic review of the literature.
Clinical Snapshot
PICO Framework
| P — Population | Patients with pemphigus vegetans (PVg), a rare variant of pemphigus vulgaris, across all ages and sexes, reported in case reports, case series, and observational studies from 1889 to January 2025 |
| I — Intervention | Systematic characterisation of clinical, histopathological, immunological features, treatment modalities (primarily oral corticosteroids and adjuvant immunosuppressants), and outcomes including remission, relapse, and mortality |
| C — Comparator | Comparison between the two clinical subtypes: Hallopeau variant (milder, pustular onset) versus Neumann variant (more severe, bullous onset); no randomised controlled trial comparator group |
| O — Outcomes | Primary: sustained remission rates and mortality by subtype. Secondary: histopathological findings (suprabasal acantholysis, eosinophilic microabscesses), immunofluorescence patterns (IgG, C3), relapse rates, treatment utilisation, and identification of triggers |
Bottom Line
This systematic review represents the most comprehensive synthesis of pemphigus vegetans (PVg) to date, consolidating data from 197 patients across 148 studies spanning 136 years. The findings confirm PVg as a rare, predominantly middle-aged condition with near-equal sex distribution, most commonly presenting at the oral mucosa and flexural sites. The Hallopeau variant carries a meaningfully better prognosis than the Neumann variant — 68% versus 52% sustained remission and 2% versus 16% mortality respectively — a distinction with direct clinical relevance for prognostication and counselling. Histological and immunofluorescence findings are consistent with pemphigus vulgaris pathophysiology, supporting shared diagnostic pathways. However, clinicians must interpret these findings with significant caution: the entire evidence base comprises case reports and series, formal risk of bias assessment is absent, and publication bias is substantial. No practice-changing treatment recommendations can be derived. For Australian practitioners, the key takeaway is heightened diagnostic vigilance — PVg should be considered in patients with vegetating flexural or oral lesions, with early biopsy and direct immunofluorescence. Management should follow established pemphigus vulgaris protocols pending disease-specific evidence, with consideration of PBS-listed rituximab for refractory cases through specialist referral.
Key Findings
P Value: Not reported
Effect Size: Descriptive proportions only; no pooled effect size calculated. Relapse occurred in 23.0% (35/152) of patients achieving remission. Oral corticosteroids used in 89.5% (145/162) of patients
Primary Outcome: Sustained remission rates and mortality stratified by clinical subtype: Hallopeau variant demonstrated 68% sustained remission (28/41) and 2% mortality (1/41); Neumann variant demonstrated 52% sustained remission (16/31) and 16% mortality (5/31)
Nnt Or Sensitivity: Not applicable in the context of this descriptive systematic review. Direct immunofluorescence sensitivity for intercellular IgG: 92.4% (121/131 patients tested). Suprabasal acantholysis on histology: 84.9% (158/186)
Confidence Interval: Not reported; no formal statistical synthesis performed
Clinical Application
Diagnostic workup (skin biopsy for histology and direct immunofluorescence) is feasible in tertiary dermatology centres. Oral corticosteroids as first-line therapy are widely available. Adjuvant immunosuppressants (azathioprine, mycophenolate mofetil) are accessible in most healthcare systems. Standardised diagnostic criteria are currently lacking, which limits consistent clinical application In Australia, pemphigus vulgaris and its variants are managed under RACGP and Australasian College of Dermatologists guidelines. Oral prednisolone is PBS-listed for autoimmune blistering disorders. Azathioprine and mycophenolate mofetil are PBS-accessible as steroid-sparing agents. Rituximab (PBS-listed for pemphigus vulgaris under specific criteria since 2021) may be relevant for refractory PVg cases, though its specific use in PVg is not addressed in this review. TGA-approved desmoglein 1 and 3 ELISA testing supports immunological profiling. Australian dermatologists should be aware that PVg may be misdiagnosed as inverse psoriasis, Hailey-Hailey disease, or condylomata, given the flexural and oral predilection. Referral to a tertiary centre with expertise in autoimmune blistering diseases is recommended for confirmed or suspected cases. Adult patients presenting with vegetating plaques or pustular lesions in flexural areas (groin, axilla) or oral mucosa, particularly where pemphigus vulgaris is suspected but clinical features are atypical. Mean age of affected patients was 52.2 years (SD 20.2) with near-equal sex distribution, suggesting broad demographic applicability
Abstract
BACKGROUND: 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. OBJECTIVES: To systematically review cases of patients with PVg and summarize clinical, histopathological, immunological features, treatment and outcomes. METHODS: 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. RESULTS: 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. CONCLUSIONS: This review highlights the need for standardized diagnostic criteria, improved immunological profiling and collaborative data collection to guide evidence-based management.
References
- 1.Kaushal, I., Herlekar, R., Mehta, H., & De, D. (2026). Pemphigus vegetans: insights from a systematic review of the literature. Clinical and Experimental Dermatology. Advance online publication. https://doi.org/10.1093/ced/llag110
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