Cicatricial Pemphigoid Medication
- Author: Anatoli Freiman, MD, FRCPC, DABD; Chief Editor: Dirk M Elston, MD more...
Medication Summary
Patients with mild localized disease may benefit from topical steroids (eg, triamcinolone [Kenalog in Orabase]) in gel-based topical agents for oral disease or in ointment-based topical steroids for cutaneous disease. Intralesional steroids can be administered as triamcinolone acetonide (Kenalog susp) 10 mg/mL injected weekly or biweekly for oral and cutaneous lesions. Patients with more extensive disease and progressive scarring require systemic therapy with prednisone and/or steroid-sparing agents, such as cyclophosphamide, azathioprine, cyclosporin, mycophenolate mofetil. Evidence from 2 small randomized controlled trials indicates that ocular cicatricial pemphigoid responds best to cyclophosphamide, while mild-to-moderate disease seems effectively suppressed by treatment with dapsone.
High-dose intravenous immune globulin has been used successfully in the treatment of cicatricial pemphigoid in patients who were refractory to other therapies. Immunosuppressive agents should be prescribed and monitored by physicians familiar with these medications. The 2002 consensus statement on cicatricial pemphigoid[1] reports expert panel opinion on the management of the disease.
Anti-inflammatory Agents
Class Summary
These agents decrease the inflammatory response.
Triamcinolone topical (Kenalog, Kenalog in Orabase)
Agent for mild disease or used as an adjuvant in patients receiving concurrent systemic therapy. Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability.
Dapsone (Avlosulfon)
DOC for ocular cicatricial pemphigoid and often beneficial in patients with oral mucosal disease. Bactericidal and bacteriostatic against mycobacteria. Mechanism of action is similar to that of sulfonamides where competitive antagonists of PABA prevent formation of folic acid, inhibiting bacterial growth.
Prednisone (Deltasone, Orasone, Sterapred)
May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.
Immunosuppressants
Class Summary
These agents inhibit immune reactions resulting from diverse stimuli.
Azathioprine (Imuran)
Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. May decrease proliferation of immune cells, which results in lower autoimmune activity.
Cyclosporine (Sandimmune, Neoral)
Demonstrated to be helpful in a variety of skin disorders. Cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions, such as delayed hypersensitivity, allograft rejection, experimental allergic encephalomyelitis, and graft-vs-host disease for a variety of organs. For children and adults, base dosing on ideal body weight.
Cyclophosphamide (Cytoxan, Neosar)
Chemically related to nitrogen mustards. As an alkylating agent, the mechanism of action of the active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells.
Mycophenolate (CellCept)
Inhibits purine synthesis and proliferation of human lymphocytes.
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