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DIF: IgA on epidermal cell surfaces IIF: Positive circulating IgA Treatment options: Steroids: topical and oral (0.5-1mg/kg) Addition of Dapsone far superior to just steroids alone (dapsone often good for treatments with neutrophilic infiltrates) Other agents: Colchichine, sulfapyridine, acitretin, PUVA Less often: MMF, adalimumab (TNf-alpha is involved in activation of neutrophilic infiltration of epidermis so may hinder further progression of IgA pemphigus) Ddx: Subcorneal pustular dermatosis (aka Sneddon Wilkinson disease) Sterile pustular lesions that erupt in cyclical patterns Like IgA pemphigus the pustular lesoins coalesce in an annular pattern and burst to form crusted plaques Similar area of distribution - groin, trunk, axilla and avoid mucous membranes Histology shows perivascular infiltration of neutrophils and spongiosis DIF negative for IgA Pemphigus foliaceous DIF is key - pemphigus foliaceous demonstrates IgG to DsG 1 Bacterial skin infections Linear IgA Disease Prognosis: Much milder and limited disease compared to classic pemphigus MANAGEMENT OF PEMPHIGUS In pemphigus, B cells produce antibodies that target desmogleins, which are proteins that connect epidermal cells together

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