Oral Manifestations of Autoimmune Blistering Diseases Treatment & Management

Updated: Feb 11, 2019
  • Author: Nita Chainani-Wu, DMD, MS, MPH, PhD; Chief Editor: William D James, MD  more...
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Treatment

Medical Care

Patients with oral manifestations of autoimmune blistering diseases should be treated conjointly with an oral medicine specialist. Furthermore, patients should have an oral prophylaxis performed by a dental hygienist or dentist prior to initiation of systemic or topical therapy. During the course of therapy, patients should have oral prophylaxis (oral hygiene) performed every 3-4 months. Additionally, they should be monitored for oral candidiasis, especially once on immunosuppressive therapy.

For patients who are treated with systemic corticosteroids, calcium and vitamin D blood levels should be monitored and supplements given if needed to reduce steroid-induced osteoporosis. Furthermore, and especially in patients with pemphigus vulgaris, a baseline bone density test should be performed.

For patients with severe disease who are treated with systemic corticosteroids, steroid-induced osteoporosis should be prevented or reduced by taking an osteoclast-mediated bone resorption inhibitor-bisphosphonate (eg, Fosamax). [27, 28]

For pemphigus vulgaris patients who do not respond to more conventional therapies, intravenous infusion of humanized monoclonal antibodies to B cells (anti-CD20, rituximab) should be discussed with the patient's primary physician, after the precaution to assess for serious infections is taken into account. [29, 30, 31, 32, 33, 34, 35]

Elderly patients who have other significant health problems (eg, diabetes mellitus, hypertension, heart diseases) may require treatment with a more conservative approach (eg, topical corticosteroids, tetracycline [36] ). The goal of treatment is to achieve disease control with low doses of medications and minimal adverse effects.

Inpatient care frequently is required for patients with the pemphigus group of diseases, particularly for those with pemphigus vulgaris and paraneoplastic pemphigus.

Transferring patients with extremely severe disease with most of the skin denuded to a burn unit for close skin care may be indicated.

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Surgical Care

Surgical care usually is not needed in treating the oral manifestations of patients with autoimmune blistering diseases.

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Consultations

Examination by pulmonary specialists is recommended for patients with severe oral lesions, especially those patients with paraneoplastic pemphigus if the patients have symptoms or signs suggestive of respiratory difficulty. Respiratory failure and death have been reported in these patients. [37, 38]

Examination by gastroenterologists is recommended for some patients with severe oral lesions to detect possible involvement of the esophagus. Dysphagia can be an associated symptom.

Examination by ophthalmologists experienced in external eye diseases is recommended for those patients with oral lesions and symptoms or signs of ocular inflammation.

Thorough examination by consulting physicians experienced in mucous membrane pemphigoid (cicatricial pemphigoid) is recommended for some patients with oral lesions that also can have genital mucosal involvement.

Care provided by oral medicine specialists or physicians experienced in the field of oral medicine is recommended for patients with severe oral disease.

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Diet

Advise patients with oral mucosal manifestations of autoimmune blistering diseases to eat a balanced diet and to avoid rough or spicy foods. Patients generally have no dietary restrictions once the disease is under control.

During periods of flare-up, soft and bland diets are preferred since it will cause less trauma to the injured tissue. Foods with strong acidity and spicy foods should be avoided. Patients with epidermolysis bullosa acquisita should avoid foods with a hard-to-chew quality since this disease tends to be exacerbated by minor trauma.

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Activity

Generally, no activity restrictions are recommended for patients with oral manifestations of autoimmune blistering diseases; however, strenuous physical activities may not be advisable for patients with epidermolysis bullosa acquisita since this disease is exacerbated by trauma.

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Complications

Monitoring treatment complications (eg, infection, osteoporosis, adrenal suppression) for patients receiving long-term immunosuppressive treatments is needed. If observed, treat complications properly.

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Prevention

Institute a combined supplement of calcium and vitamin D for patients treated with systemic corticosteroid for longer than 1 month to prevent osteoporosis. The guideline for dosage and frequency of this supplement is stated in the 1996 recommendations established by the American College of Rheumatology Task Force. [39]

Institute a combined regimen of a non-alcohol–based mouthwash (Biotene mouthwash) and a weekly dose of systemic antifungal medication for patients at risk for oral candidiasis.

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Long-Term Monitoring

Regular follow-up care for patients with oral and skin involvement by their dermatologist and oral medicine specialist is recommended when the disease is active and during the tapering of medication when the disease is in remission.

Patients with oral disease alone should receive follow-up care from an oral medicine specialist, otherwise poor oral hygiene will interfere with treatment outcome. Furthermore, complications from poor oral care could lead to periodontal disease and early teeth loss.

During the active disease stage, patient follow-up care every 4-6 weeks is recommended. Patients should be monitored for oral yeast infection. During the clinical remission stage, patient follow-up care every 6 months is recommended.

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