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.
Kennedy
Jeremy Scott
JS
San Antonio Military Medical Center South, Lackland Air Force Base, TX 78236, USA.
Devillez
Richard L
RL
Henning
Jeffrey Scott
JS
D000287:Administration, Topical; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use*; D001379:Azathioprine / Q000627:therapeutic use; D003879:Dermatologic Agents / Q000627:therapeutic use; D004351:Drug Resistance; D004359:Drug Therapy, Combination; D000068800:Etanercept; D005145:Face / Q000473:pathology; D005260:Female; D005881:Gingiva / Q000473:pathology; D006801:Humans; D007074:Immunoglobulin G / Q000627:therapeutic use*; D008875:Middle Aged; D010159:Palate / Q000473:pathology; D010390:Pemphigoid, Benign Mucous Membrane / Q000188:drug therapy* / Q000473:pathology; D018124:Receptors, Tumor Necrosis Factor / Q000627:therapeutic use*; D013256:Steroids / Q000627:therapeutic use; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors*
D000893:Anti-Inflammatory Agents; D000894:Anti-Inflammatory Agents, Non-Steroidal; D003879:Dermatologic Agents; D007074:Immunoglobulin G; D018124:Receptors, Tumor Necrosis Factor; D013256:Steroids; D014409:Tumor Necrosis Factor-alpha; D001379:Azathioprine; D000068800:Etanercept