PVeg is rare and makes up an estimated 2%-5% of the total cases of pemphigus. Although this disease can affect people of all age groups, it occurs primarily during middle age with a median onset of 40-60 years. It affects males and females equally.
PVeg has been divided into two subtypes depending on clinical presentation and disease course:
- The Neumann type is characterized by flaccid bullae and erosions in intertriginous areas that develop overlying vegetations. This subtype is more common, with a clinical course that parallels PV.
- The Hallopeau type has a more benign course and is characterized by erupting crops of pustules that evolve into crusted plaques. It may remit spontaneously.
L10.1 – Pemphigus vegetans
81285006 – Pemphigus vegetans
- Generalized PV – Similar in presentation to Neumann type but not limited to intertriginous areas and without vegetating plaques.
- Paraneoplastic pemphigus – Associated with malignancy, most commonly non-Hodgkin lymphoma; mucous membranes are the site of most severe involvement.
- IgA pemphigus – Mucous membranes are spared; immunoglobulin A (IgA) autoantibodies versus IgG in PVeg.
- Drug-induced pemphigus – Most commonly captopril and D-penicillamine implicated; a precise drug history is critical, as these can be difficult to distinguish, especially if only oral lesions are present. Pemphigus foliaceus is more common than PV in cases of drug-induced pemphigus.
- Pemphigus foliaceus (PF) – Mucous membranes are spared; scales and crusts over erythematous skin in seborrheic areas.
- Pemphigus erythematosus (also known as Senear-Usher syndrome) is a localized variety of PF largely confined to the malar region and seborrheic sites.
- Fogo selvagem is an endemic form of PF found in rural Brazil.
- Pemphigoid – IgG that targets hemidesmosomes; results in subepidermal blisters. In PVeg, the blisters are intraepidermal. Oral involvement occurs in only 10%-35% of cases of pemphigoid. Nikolsky sign is negative in pemphigoid.
- Bullous impetigo – Flaccid bullae; acantholysis on histology, but direct immunofluorescence (DIF) is negative. Blisters often become pustular.
- Pemphigoid vegetans – A rare variant of pemphigoid with vegetative plaques also found in intertriginous areas; has the immunopathological features of pemphigoid.
- Pyostomatitis vegetans – Intraoral blistering most often associated with ulcerative colitis, but may also be associated with other inflammatory bowel diseases; clinically similar to PVeg and only distinguishable by characteristically negative immunofluorescence findings for autoantibodies.
- Linear IgA dermatosis – Annular configuration of bullae; subepidermal blisters with DIF showing IgA at dermal-epidermal junction.
- Dermatitis herpetiformis – Positive for antiendomysial and anti-transglutaminase antibodies; classically, crops of vesicles and urticarial plaques develop on extensor surfaces and gluteal-sacral and scapular regions; DIF shows IgA in the dermal papillae. Extremely itchy.
- Hailey-Hailey disease (benign familial pemphigus) – May have similar clinical features and demonstrate acantholysis on histology but will lack the immunofluorescence findings of PVeg.
- Darier disease (keratosis follicularis) – Often in a seborrheic distribution with acantholysis on histology. As an autosomal dominant disease, often presents earlier with a family history and other clinical findings.
- Transient acantholytic dermatosis (Grover disease) – May have similar clinical findings, but will never have oral involvement and will lack findings on DIF.
- Inverse psoriasis