Therapeutic Approaches to Patients with Mucous Membrane Pemphigoid




The therapeutic approach to mucous membrane pemphigoid is site specific, with the goal of preserving function for patients with chronic and treatment-resistant disease. The involvement of certain mucosal sites (ie, ocular, laryngeal, esophageal, nasopharyngeal, and anogenital) is high risk and warrants more aggressive intervention. Control of the disease must be balanced with minimizing the sequelae of long-term exposure to systemic glucocorticosteroids and/or other immunosuppressives. Timely interventions and multidisciplinary management are essential in preventing disability.


This article summarizes the treatment of mucous membrane pemphigoid (MMP), a treatment-resistant disease for which the goal of therapy is control of symptoms and preservation of function. The arsenal of therapeutics is roughly the same as that for bullous pemphigoid with additional emphasis on site-directed therapy as the guiding principle of management.


Vigilant review of symptoms, examination of mucous membranes and skin to determine all sites of involvement, and prompt referral to subspecialists (ie, ophthalmologists, otolaryngologists and so forth) to assist in evaluation and management of affected sites, can make the difference between independent functioning and disability for patients with MMP, especially those with ocular and laryngeal involvement. The involvement of certain mucosal sites (ie, ocular, genital, nasopharyngeal, esophageal, and laryngeal mucosal sites) is considered high risk and warrants more aggressive intervention. As in the treatment of any autoimmune disease, effective treatment must be reconciled with efforts to minimize the adverse effects of exposure to systemic glucocorticosteroids and/or other immunosuppressives.


Approach to patients with low-risk disease


Low-risk disease (eg, involvement of only the oral mucosa and/or skin sites with less tendency and/or clinical significance of scarring) is managed first with topical corticosteroids of moderate to high potency. Available forms include mouthwash (dexamethasone swish-and-spit) and topical corticosteroid gels or ointments. To facilitate adsorption, gels and ointments can be used under occlusion with oral, insertable, vinyl, prosthetic devices (ie, dental trays). Avoidance of toothpastes containing sodium lauryl sulfate and mouthwashes containing alcohol may further aid in the control of symptoms. Treatment may be escalated with intralesional glucocorticosteroid injections and systemic therapies, such as dapsone, low morning doses of prednisone, or the prednisone in combination with azathioprine or mycophenolate mofetil ( Table 1 ).



Table 1

Complications and treatment by mucosal site




































Site Possible Complications Site-Specific Therapy
Mouth

  • •

    Adhesions between tongue and floor of mouth and around uvula and tonsils


  • •

    Dental complications:



    • ○

      Gingival loss


    • ○

      Caries


    • ○

      Periodontal ligament damage


    • ○

      Loss of bone mass


    • ○

      Loss of teeth





  • Mild:


  • •

    Initial treatment with topical glucocorticosteroids



    • ○

      Moderate to high potency (class I–III steroids) gels or ointments bid/qid


    • ○

      Mouthwash (dexamethasone 100 g/mL, 5 mL per rinse) used in a swish-and-spit regimen for 5 min bid/tid


    • ○

      Under occlusion (ie, beneath prosthetic vinyl dental trays)


    • ○

      Avoidance of toothpastes containing sodium lauryl sulfate


    • ○

      Avoidance of mouthwashes containing alcohol


    • ○

      Topical agents for pain (eg, lidocaine or benzocaine [beware potential complications])



  • •

    Topical calcineurin inhibitor (eg, tacrolimus)


  • •

    Intralesional glucocorticosteroids




  • Moderate:


  • •

    Dapsone (25–200 mg/d)


  • •

    Low morning doses of prednisone (0.5 mg/kg or 20–40 mg/d with or without azathioprine [2.0–2.5 mg/kg/d], mycophenolate mofetil [1.0 to 2.5 g/d], or cyclophosphamide [1.0 to 2.0 mg/kg/d])




  • Severe:


  • •

    Higher dose of prednisone (1 mg/kg or 60 mg/d with or without azathioprine [2.0–2.5 mg/kg/d], mycophenolate mofetil [1.0–2.5 g/d], or cyclophosphamide [1.0–2.0 mg/kg/d])




  • Alternatives/Miscellaneous:


  • •

    Thalidomide, tetracycline/niacinamide, IVIG, plasmapheresis, rituximab


  • •

    Surgery for scarring, tissue loss

Eye

  • •

    Painful, erosive, conjunctivitis


  • •

    Conjunctival scarring


  • •

    Shortened fornices


  • •

    Loss of goblet cells


  • •

    Decrease in tear mucus content, unstable tear film


  • •

    Symblepharon


  • •

    Ankyloblepharon


  • •

    Ectropion


  • •

    Trichiasis


  • •

    Corneal irritation


  • •

    Superficial punctate keratinopathy


  • •

    Corneal neovascularization


  • •

    Corneal ulcers


  • •

    Scarring, occlusion, secondary infection of the tear ducts


  • •

    Blindness



  • •

    Epilation of eyelashes for trichiasis


  • •

    Topical glucocorticosteroids per decision of ophthalmologist




  • Mild to Moderate:


  • •

    Dapsone (25–200 mg/d)


  • •

    Low morning doses of prednisone (0.5 mg/kg or 20–40 mg/d with or without azathioprine [2.0–2.5 mg/kg/d], mycophenolate mofetil [1.0–2.5 g/d], or cyclophosphamide [1.0–2.0 mg/kg/d])




  • Severe:


  • •

    Higher dose of prednisone (1 mg/kg or 60 mg/d with or without azathioprine [2.0–2.5 mg/kg/d], mycophenolate mofetil [1.0–2.5 g/d], or cyclophosphamide [1.0–2.0 mg/kg/d])


  • •

    IVIG (2 g/kg of body weight) over 2–3 days every 2–6 weeks for 4–6 months)


  • •

    Biologic agents:



    • ○

      Anti-TNF agents (eg, etanercept, infliximab)


    • ○

      Anti-CD 20 (rituximab)





  • Alternatives/Miscellaneous:


  • •

    Subconjunctival mitomycin


  • •

    Surgical interventions per ophthalmology

Nose

  • •

    Scarring


  • •

    Tissue loss




  • Mild:


  • •

    Irrigation with isotonic saline or tap water 2 or 3 times a day


  • •

    Nasal emollients


  • •

    Topical corticosteroids (eg, nasal sprays, inhalers)




  • Mild, Moderate, and/or Severe:


  • •

    The same as outlined for ocular treatments

Larynx

  • •

    Chronic erosions, edema, scarring leading to:



    • ○

      Supraglottic stenosis


    • ○

      Airway compromise





  • Mild, Moderate, and/or Severe:


  • •

    The same as outlined for ocular treatments


  • •

    Tracheostomy for airway compromise

Esophagus

  • •

    Esophageal dysfunction and reflux, causing:



    • ○

      Exacerbation of laryngeal disease


    • ○

      Bronchospasm



  • •

    Stricture formation


  • •

    Weight loss


  • •

    Aspiration




  • Mild, Moderate, and/or Severe:


  • •

    The same as outlined for ocular treatments


  • •

    Esophageal dilation

Anogenital region

  • •

    Urethral stricture


  • •

    Vaginal and/or anal stenosis


  • •

    Secondary infection



  • •

    Topical corticosteroids


  • •

    Topical calcineurin inhibitor (tacrolimus)




  • Mild, Moderate, and/or Severe:


  • •

    The same as outlined for ocular treatments


  • •

    Surgical interventions per urology/gynecology

Skin

  • •

    Scarring



  • •

    Topical corticosteroids


  • •

    Topical calcineurin inhibitors




  • Mild, Moderate, and/or Severe:


  • •

    The same as outlined for ocular treatments

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Feb 12, 2018 | Posted by in Dermatology | Comments Off on Therapeutic Approaches to Patients with Mucous Membrane Pemphigoid

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