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Therapeutic Approach

Mucous membrane pemphigoid (MMP) is a rare, scarring, mucosal-predominant subepidermal immunobullous disease characterized by autoantibodies directed against structural proteins in epidermal basement membrane. MMP can affect the oral, ocular, nasopharyngeal, hypopharyngeal, laryngeal, esophageal, and/or the anogenital mucosa with or without cutaneous involvement. Therapy is determined in part by the sites of involvement, the potential for complications (eg, loss of vision, loss of airway, etc.), and the kinetics and relative severity of disease. Therapy may consist of topical, local, and/or systemic treatments. Control of disease is important in that MMP-induced scarring can be prevented, but not reversed.

Our Approach to Management of MMP

“Low risk” disease (eg, involvement of only the oral mucosa and/or skin—sites with less tendency for severe scarring) is managed first with topical corticosteroids of moderate to high potency (typically ointments or gels). To facilitate gingival adsorption of these agents, they are often used under occlusion with vinyl prosthetic dental trays. Avoidance of toothpastes containing sodium lauryl sulfate and mouthwashes containing alcohol further aid in the control of symptoms. Good dental hygiene is key. Treatment may be escalated with intralesional glucocorticosteroid injections and/or systemic therapies, such as dapsone (25-200 mg/d) and/or low morning doses of prednisone (10-40 mg/d). For patients with “high risk” disease (eg, involvement of ocular, nasopharyngeal, hypopharyngeal, laryngeal, esophageal, and/or anogenital mucosae), systemic glucocorticosteroids (ie, prednisone at doses of 1 mg/kg/d) in combination with glucocorticosteroid sparing agents (eg, mycophenolate mofetil [1.0-3.0 g/d], methotrexate 15-25 mg weekly, azathioprine [50-150 mg/d], cyclophosphamide [1.0-2.0 mg/kg] or rituximab [375 mg/m2 IV weekly × 4, consider repeating in 4 to 6 months]) represent mainstays of treatment. Among these agents, rituximab (375 mg/m2 IV weekly × 4, consider repeating in 4 to 6 months) is moving to the fore. Although the only randomized blinded controlled study for MMP demonstrated efficacy of cyclophosphamide, we prefer rituximab due to its better short-term and long-term side effect profiles. Once MMP is brought under control, daily prednisone is tapered gradually over approximately 6 months, and the patient is maintained on an alternate agent alone for an additional 12 to 24 months (the latter time period being particularly relevant for patients with ocular disease). In cases of refractory “high risk” disease, intravenous immunoglobulin (IVIG, 2 g/kg over 2 to 3 days administered every 2 to 6 weeks) is commonly used, often in combination with rituximab. Recent case series of tofacitinib (5 mg BID or 11 mg Extended-Release Tab QD), or baricitinib 2 mg daily in combination with methotrexate (5-15 mg/wk) offer promise for patients with recalcitrant disease. In managing “high risk” patients, it is important to consider that those on long-term prednisone and other immunosuppressive treatments may require prophylaxis for Candida, viral, and/or Pneumocystis infections as well as therapies for prevention of osteoporosis (eg, calcium, vitamin D, and/or bisphosphonates). All patients with MMP require careful application of local ...

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