Pemphigus vulgaris is a severe chronic bullous B-cell-mediated autoimmune disease in which autoantibodies develop into antigens within the desmosome-tonofilament junction of the intercellular bridges. Such autoantibodies fix complement and initiate inflammation, which causes a suprabasilar split (intraepithelial blister) as the primary pathogenesis.
Autoimmunity. Desmoglein 1 and 3 are the main target antigens.
- Four classical varieties of pemphigus are recognized: vulgaris, vegetans, foliaceus, and erythematosus.
- Recently, two additional forms of the disease have been described: drug-induced pemphigus and paraneoplastic pemphigus, which usually affect patients with lymphoreticular malignancies.
- Pemphigus vulgaris is the most common variant, representing 90–95% of cases. Pemphigus vulgaris usually presents with painful skin and/or oral ulcers. More than 70% of pemphigus vulgaris cases begin with oral involvement.
- Oral lesions are characterized by the formation of bullae, which rapidly rupture, leaving painful erosions with a tendency to extend peripherally. The buccal mucosa, labial mucosa, palate, tongue, floor of the mouth, and gingiva are often involved.
- The skin lesions present as flaccid bullae that rupture quickly, leaving persistent eroded areas. Nikolsky’s sign (Gently stroking the mucosa can cause a vesicle or bulla to appear) is positive. Any skin area may be involved, although the intertriginous regions, umbilicus, trunk, and scalp are the most common sites affected. Lesions may develop on other mucosae (conjunctivae, nose, larynx, pharynx, genitals, anus).
Histopathological and cytological examination, direct and indirect immunofluorescence.
Cicatricial pemphigoid, bullous pemphigoid, linear IgA disease, epidermolysis bullosa acquisita, toxic epidermal necrolysis, primary herpetic gingivostomatitis, erythema multiforme, erosive lichen planus, aphthous ulcers.
Oral-only pemphigus vulgaris responds well to systemic corticosteroid regimen I . Approximately 70% of cases of pemphigus vulgaris with both oral and skin lesions also respond to systemic corticosteroid regimen I. The remaining 30% of cases of this type of pemphigus vulgaris respond incompletely to prednisone and require the addition of either rituximab, cyclophosphamide or azathioprine.