[Mucous membrane pemphigoid: a review]

Abstract

Background: Mucous membrane pemphigoid is a rare autoimmune bullous disorder. Numerous treatment regimens have been proposed in the literature.

Objective: To assess the efficacy and tolerance of treatment regimens proposed in mucous membrane pemphigoid (MMP), from a systematic review of the literature.

Methods: Randomized control trials have been identified using the PubMed and Embase databases up to April 2009. Uncontrolled prospective and retrospective studies have also been analyzed.

Results: Literature analysis confirms that clinical and therapeutic trials are very uncommon in MMP; only retrospective series or case reports are available and have been analyzed. Therefore, the level of evidence is usually weak. Twenty-four series have been analyzed in this review. Dapsone remains the first line treatment in non-ocular forms of MMP. Sulfasalazine or cyclins can be used when dapsone is not tolerated or effective. Corticosteroids can be used to control inflammatory flares of the disease. Immunosuppressants are not used as the first line of treatment and can be added to anti-inflammatory drugs for a better control of MMP. Cyclophophamide or mycophenolate mofetil can be used, especially in the elderly. In ocular forms of the disease, the severity and chronicity of ocular involvement is the main therapeutical target. Non-scarring conjunctivitis can be treated by dapsone monotherapy. Ocular flares of the disease can be treated with systemic corticosteroids or cyclophosphamide. Many immunomodulating drugs are under evaluation. Intravenous immunoglobulins, etanercept or rituximab can be proposed when cyclophosphamide is not able to control the disease.

Conclusion: Data from the literature did not allow identifying the best therapeutic regimen, mainly because of the lack of prospective comparative studies. Dapsone remains the first line treatment in MMP. Immunosuppressive or immunomodulating drugs should be discussed patient by patient.