Pemphigus vegetans - Skin
The essentials of diagnosing pemphigus disorders include the following:
- Groups of bullae that remit and relapse, often preceded by erosion, ulceration, and blistering of mucous membranes
- Positive Nikolsky's sign
- Biopsy of lesions: histopathology shows acantholysis
- Diagnostic confirmation of autoantibodies via direct immunofluorescence
The incidence of pemphigus vulgaris is approximately one per million people, with the disease being more common among people of Ashkenazi Jewish and Mediterranean descent. Pemphigus vegetans makes up an estimated 2% to 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.
Pemphigus vegetans has been divided into two subtypes relating to clinical presentation and disease course: the Neumann type and the Hallopeau type. In the Neumann type, lesions are classically flaccid bullae and erosions in intertriginous areas that progress into vegetations. The Neumann type is more common, with a course very similar to that of pemphigus vulgaris. It usually requires high-dose systemic corticosteroids to treat; relapses are frequent. The Hallopeau type has a more benign course and is characterized by crops of pustules erupting and eventually progressing to dark plaques. Lower doses of systemic corticosteroids can be used, and some cases may even remit spontaneously. Oral mucosal involvement is nearly always present in both subtypes, as is the development of hyperpigmented vegetative plaques with thickened granulation tissue at the lesion's rim.
L10.1 – Pemphigus vegetans
81285006 – Pemphigus vegetans
Paraneoplastic pemphigus – associated with malignancy, most commonly non-Hodgkin's lymphoma; mucous membranes are the site of most severe involvement.
IgA pemphigus – mucous membranes are spared; IgA autoantibodies versus IgG in pemphigus vegetans.
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 pemphigus vulgaris 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.
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 pemphigus vegetans and only distinguishable by characteristically negative IF 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, gluteal-sacral, and scapular regions; DIF shows IgA in the dermal papillae. Extremely itchy.