An 18-year-old woman presented to the dermatology clinic for evaluation of recurrent skin, nail, and oral mucosal abnormalities. At age 4 years, she developed white oral plaques that resolved with oral nystatin. Approximately 6 months later, the oral plaques recurred, her toenails became thickened and yellowed, and red plaques appeared on both feet and lower legs. After 3 months of daily oral itraconazole, her skin, nail, and oral mucosal abnormalities resolved for approximately 1 year. Over the following years, she received intermittent 3- to 6-month courses of daily itraconazole for recurrent skin, nail, and oral mucosal abnormalities, which typically recurred within 6 to 12 months of discontinuing itraconazole. Her last dose of itraconazole was approximately 2 years prior to presentation. On physical examination, the patient had white, moist, nonadherent plaques in the oral mucosa and moist red fissures at bilateral oral commissures (Figure, left panel). She also had thickened, yellowed toenails and scaly erythematous plaques on her lower legs and feet bilaterally (Figure, right panel). Results of complete blood cell count with differential were normal, as were results of measurement of serum IgG, IgA, IgM, and IgE levels and analysis of CD3, CD4, and CD8 T-cell subsets. Potassium hydroxide wet mount preparation of skin scrapings from the lower extremities demonstrated fungal elements. Fungal culture of the skin scrapings grew Candida albicans.
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Chronic mucocutaneous candidiasis (CMC)
C. Restart daily oral itraconazole
The key to the correct diagnosis is recognition that recurrent fungal infections of the oropharynx, skin, and nails are characteristic of CMC. Choices A and B are incorrect because antibiotics and steroid creams are not treatments for candidiasis and may worsen this condition. Although terbinafine (choice D) is an antifungal medication, it is not first-line therapy for CMC.
Chronic mucocutaneous candidiasis is a primary immunodeficiency disorder characterized by persistent or recurrent noninvasive infections of the skin, nails, oral cavity, and genital mucosa with Candida species, typically C albicans.1
The differential diagnosis of CMC includes other T-cell deficiency diseases that cause chronic candidiasis such as HIV, severe combined immunodeficiency, CARD9 (caspase recruitment domain-containing protein 9) deficiency, CD25 deficiency, and hyperimmunoglobulin E syndrome. Unlike CMC, these conditions are typically associated with invasive Candida infections.2
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CME Disclosure Statement: Unless noted, all individuals in control of content reported no relevant financial relationships. If applicable, all relevant financial relationships have been mitigated.
Corresponding Author: Xiao-Yong Man, MD, PhD, Department of Dermatology, Second Affiliated Hospital, Zhejiang University School of Medicine, 88 JF Rd, Hangzhou, Zhejiang 310000, China (email@example.com).
Published Online: August 18, 2022. doi:10.1001/jama.2022.14460
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patient for providing permission to share her information.
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