Medication Summary
Patients with mild localized mucous membrane pemphigoid (MMP) (cicatricial pemphigoid) 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, or mycophenolate mofetil. Evidence from two small randomized controlled trials indicates that ocular mucous membrane pemphigoid responds best to cyclophosphamide, while mild-to-moderate disease seems effectively suppressed by treatment with dapsone.
Rituximab is a therapeutic option for refractory cases. Maley and colleagues retrospectively studied 24 patients treated with rituximab and 25 with conventional immunosuppressants. One hundred percent of patients treated with rituximab achieved control of the disease, compared with 40% of the patients treated with conventional therapy. [17]
High-dose intravenous immune globulin has been used successfully in the treatment of mucous membrane pemphigoid in patients whose disease was refractory to other therapies. This therapy can be very helpful in patients who have severe adverse effects from conventional therapy. [18] Immunosuppressive agents should be prescribed and monitored by physicians familiar with these medications. The 2002 consensus statement on mucous membrane pemphigoid reports expert panel opinion on the management of the disease. [1]
Anti-inflammatory Agents
Class Summary
These agents decrease the inflammatory response.
Triamcinolone topical (Kenalog, Kenalog in Orabase)
Triamcinolone topical is an agent for mild disease or is used as an adjuvant in patients receiving concurrent systemic therapy. It decreases inflammation by suppressing the migration of polymorphonuclear leukocytes and reversing capillary permeability.
Dapsone (Avlosulfon)
Dapsone is the drug of choice for ocular mucous membrane pemphigoid and is often beneficial in patients with oral mucosal disease. It is bactericidal and bacteriostatic against mycobacteria. Its 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)
Prednisone 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)
Azathioprine antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. It may decrease the proliferation of immune cells, which results in lower autoimmune activity.
Cyclosporine (Sandimmune, Neoral)
Cyclosporine has demonstrated to be helpful in a variety of skin disorders. It is a 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)
Cyclophosphamide is 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)
Mycophenolate inhibits purine synthesis and the proliferation of human lymphocytes.
Antineoplastics, Anti-CD20 Monoclonal Antibodies
Class Summary
Antineoplastic agents act by inhibiting the key factors responsible for neoplastic transformation of cells. The agents in this class target specific antigens in carcinoma cells and induce cytotoxicity.
Rituximab (Rituxan)
Rituximab is a genetically engineered chimeric murine/human monoclonal antibody directed against the CD20 antigen found on the surface of normal and malignant B lymphocytes. The antibody is an IgG1 kappa immunoglobulin containing murine light- and heavy-chain variable region sequences and human constant region sequences.
Immune Globulins
Class Summary
Intravenous immunoglobulins have proven beneficial in achieving rapid disease control in patients with immunobullous diseases.
Immune globulin IV (IGIV) (Bivigam, Carimune, Carimune NF)
Immune globulin intravenous consists of IgG collected from a pool of thousands of blood donors (virus-free), thus providing a wide range of immunologically different IgG. Theoretically, they bind and neutralize pathogenic autoantibodies.
Cost and availability limit its use. Immune globulin intravenous is expensive. It is generally used in resistant and severe bullous diseases in addition to immunosuppressive therapy or as monotherapy in patients with contraindications for immunosuppressive drugs.
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Ocular manifestations of cicatricial pemphigoid (mucous membrane pemphigoid) include symblepharon, demonstrated in this photograph by the tethering of the lower lid to the cornea.
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In a patient with more advanced ocular scarring, note the thickening of the lid margins, shortening of the conjunctival sulcus, and scarring. The eyelashes have been epilated after entropion developed.
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By direct immunofluorescence, a linear band of immunoreactants at the epidermal-dermal junction is demonstrated by using a fluorescein-tagged antibody specific for human immunoglobulin G.
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With advanced disease, ankyloblepharon (a fixed globe) develops.
