Chronic Mucocutaneous Candidiasis Treatment & Management

Updated: Apr 17, 2017
  • Author: Blanca Anais Estupiñan; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Medical Care

Management can be difficult, and relapse is common following discontinuation of therapy. Topical therapies are not usually effective in patients with chronic mucocutaneous candidiasis (CMC). Treatment of oral involvement in CMC can be aided by therapy with clotrimazole troches or oral nystatin solution. Treatment falls into three main categories: antifungal agents, immunologic therapies, and combination therapy.

Systemic antifungal therapy is the mainstay of CMC therapy. It may be used alone or in combination with an immunomodulatory agent. The drawbacks of systemic antifungal therapy include the risk of adverse effects or toxicity, a failure to correct the underlying immune deficiency, relapse following the cessation of therapy, and antifungal resistance to some antifungal agents. It is estimated that 40% of patients receiving long-term antifungal treatment develop drug resistance, although lower frequency is observed with intermittent therapy. [28] Patients with resistance to antifungals usually present with more severe phenotypes such as systemic infections and recurrent pneumonia. First-line antifungals include fluconazole, itraconazole, and posaconazole, while second-line therapy includes voriconazole, echinocandins, terbinafine, and liposomal amphotericin B.

Several immunologic therapies have been proposed in an effort to correct the underlying immune deficiency in persons with CMC. The most widely studied treatment is the use of transfer factor. [31] Transfer factor is a cell-free protein extracted from the T lymphocytes of Candida-immune donors. Although the precise mechanism is unknown, it has been shown to transfer delayed-type hypersensitivity reactions to patients previously anergic to candidal skin testing. Candida-specific cell immunity may be transferred by this approach. It is not effective in all cases. Long-term remissions have occurred when combined with antifungal medications. Patients with CMC associated with autoimmune manifestations due to STAT1 mutations may show improvement with JAK1/2 tyrosine kinase inhibitors such as ruxolitinib. [32, 33] Novel therapies such as JAK1/2 inhibitors have demonstrated sufficient potency to attenuate the increased STAT1 phosphorylation in CD4+ T lymphocytes, while enhancing IL-production, resulting in improvement of both the immunodeficiency and autoimmune disease processes. [34]

Other systemic immunologic treatments include intravenous immunoglobulin G (IgG) or granulocyte macrophage colony-stimulating factor (GM-CSF) infusions and interferon-alfa.

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Consultations

Refer patients to an endocrinologist if screening laboratory test results suggest an associated endocrine abnormality.

If familial chronic mucocutaneous candidiasis (CMC) is suspected, consultation with a geneticist should be obtained.

Patients with recurrent infections or pneumonia should be referred to an immunologist.

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Long-Term Monitoring

Baseline studies and yearly screening for associated endocrinopathy should be performed. Long-term follow-up is necessary for identifying and addressing accompanying disorders.

Some authors suggest screening angio-MRI for all patients with diagnosed CMC to rule out aneurysm, but this is not a universal recommendation. [6] Owing to chronic mucocutaneous candidiasis (CMC) and resultant chronic mucocutaneous inflammation, patients are at increased risk of skin and ear, nose, and throat cancers. [35] Sequential biopsies of the esophagus to screen for tumors in patients presenting with recurrent esophagitis and associated dysphagia is recommended by some authors. [28]

If the disease flares, patients may need to be seen on an urgent basis, particularly after a course of antifungals has been discontinued.

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