Summary
Pemphigus is a group of chronic autoimmune skin diseases characterised by blister formations on the outer layer of the skin and the mucous membranes. Three clinical forms have been characterised, one of which is superficial pemphigus, including the seborrheic, erythematosus, foliaceous and herpetiform variants. Prevalence of superficial pemphigus (corresponding to 25% of all pemphigus cases) is estimated at approximately 1/83,330. Superficial pemphigus only affects the outer layer of the skin and clinical presentation varies. Mucosal lesions are absent and skin lesions consist of superficial crusts or, in some cases, erythemato-squamous plaques, which can be pruritic (evocative of systemic lupus erythematosus). The involvement can be generalised or restricted to seborrheic areas (such as the face, back, chest). The exact causes of the disease are unknown. Auto-antibodies directed against desmosome components cause acantholysis and intra-epidermal cleft. In superficial pemphigus, auto-antibodies bind to desmogleine 1 only. Diagnosis is confirmed by histopathological analysis, showing cleavage in the granulous layer, with sub-corneal blister formation. Acantholysis is a constant feature. Direct immunofluorescence test shows IgG and/or C3 complement deposits at the cell surface of keratinocytes. Systemic steroid therapy is the therapy of choice. It can be combined with immunosuppressant drugs or local steroid therapy. Superficial pemphigus has a better prognosis than pemphigus vulgaris (see this term). * Author: Orphanet (November 2007) *.
