Recalcitrant cicatricial pemphigoid treated with the anti-TNF-alpha agent etanercept

Cicatricial pemphigoid (mucous membrane pemphigoid) is a group of chronic disorders characterized by recurrent subepithelial blisters and scarring, predominantly of the mucous membranes. The authors report the case of a 63-year-old female with a 20-year history of cicatricial pemphigoid who had failed treatment with cytoxan, dapsone, systemic steroids and minocycline. She achieved complete clinical remission with the addition of etanercept 50 mg twice a week with azathioprine 150 mg daily. This patient has remained clear for two years with this combination and has tolerated a tapering of the azathioprine from the initial dose of 250 mg. Etanercept is a recombinant human dimeric fusion protein which acts as a competitive inhibitor of TNF-alpha by binding to both soluble and receptor-bound molecules of TNF-alpha. There have been a few reports of successful use of TNF-alpha inhibitors in cicatricial pemphigoid. This report will discuss the possible mechanism of action of the addition of TNF-alpha inhibitors as a steroid-sparing agent in these patients.