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Chronic Mucocutaneous Candidiasis

  • Author: Neil Sandhu, MD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Jun 30, 2016
 

Background

Chronic mucocutaneous candidiasis (CMC) refers to a heterogeneous group of disorders characterized by recurrent or persistent superficial infections of the skin, mucous membranes, and nails with Candida organisms, usually Candida albicans. These disorders are confined to the cutaneous surface, with little propensity for systemic dissemination. CMC does not represent a specific disease, but rather a phenotypic presentation of a spectrum of immunologic, endocrinologic, and autoimmune disorders. The unifying feature of these heterogeneous disorders is impaired cell-mediated immunity against Candida species.

The image below depicts CMC of the nails.

Thickened, fragmented, hyperkeratotic nails and er Thickened, fragmented, hyperkeratotic nails and erythematous periungual skin. Courtesy of Walter Reed Army Medical Center.
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Pathophysiology

C albicans is an opportunistic yeast that is part of the normal flora of the gastrointestinal tract, skin, and mucous membranes. The fungus can exist in the yeast, the mycelial (pseudohyphal), or the chlamydospore phase. Invasive disease is rare; however, when it occurs, it is usually associated with mycelial elements. Several host factors are important in defending against infection by candidal organisms. Healthy, intact skin that continuously desquamates and regenerates is usually an effective initial barrier. An intact immune system is critical for keeping this opportunistic organism at bay.

CMC is associated with a defect in cell-mediated immunity that may either be limited to Candida antigens or be part of a more general immune abnormality. Recent data suggest alterations in cytokine production in response to Candida antigens. These alterations include decreased interleukin 2 and interferon-gamma levels (TH 1 cytokines) and increased interleukin 10 levels in some studies.[1, 2] Patients who lack T-cell immunity (eg, those with severe combined immune deficiency syndrome or DiGeorge syndrome) or patients with severely impaired T-cell function (eg, patients with AIDS) are susceptible to chronic candidal infections. Defects in humoral immunity are not commonly observed in patients with CMC, and patients with antibody deficiencies are not particularly prone to candidiasis.

A study of peripheral-blood mononuclear cells from 14 patients with autosomal dominant CMC found poor production of interferon-gamma, interleukin-17, and interleukin-22. Gene sequencing identified mutations in signal transducer and activator of transcription 1 (STAT1), leading to defective immune responses in type 1 and type 17 helper T cells.[3, 4, 5]

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Epidemiology

Race

No racial predilection is reported for CMC, although APECED is most prevalent in Finnish, Sardinian, and Iranian Jewish populations.

Sex

The male-to-female ratio for CMC is equal.

Age

CMC usually manifests in infancy or early childhood (60-80% of cases), with a mean age of onset of 3 years. Delayed or adult onset of the disease is reported and can be associated with thymoma, myasthenia gravis, and bone marrow abnormalities.

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Prognosis

CMC is a chronic disease, and recurrent and relapsing superficial infections with Candida organisms should be expected. Life expectancy is generally normal but significant morbidity is associated with the chronic nail and mucocutaneous infections and associated endocrine and/or autoimmune disease. In rare cases, premature death occurs secondary to disseminated Candida infection, sepsis, pneumonia, or mycotic aneurysms.

CMC is not associated with a high degree of mortality because disseminated invasive candidal infections are rare. In isolated CMC, the prognosis is good; however, the significant morbidity is related to chronic and persistent skin, nail, and mucous membrane candidal infections. The risk of mycotic aneurysms, while low, remains a real possibility.[6] In a subset of patients, malignant thymomas or cancers of the oral cavity and digestive tract may occur. Patients with autoimmune polyendocrinopathy-candidosis-ectodermal dystrophy (APECED) have significant morbidity from endocrinopathies or other autoimmune diseases associated with this condition. Several cases of Pneumocystis carinii pneumonia in patients with CMC are reported in the literature.

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Patient Education

Patients should be aware of the chronic, recurrent nature of their disease and the current paucity of effective long-lasting therapies.

For excellent patient education resources, visit eMedicineHealth's Infections Center. Also, see eMedicineHealth's patient education article Candidiasis (Yeast Infection).

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Contributor Information and Disclosures
Author

Neil Sandhu, MD Dermatologist (Medical/Cosmetics) and Mohs Surgeon, Gulf Coast Dermatology

Neil Sandhu, MD is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Jeffrey J Miller, MD Associate Professor of Dermatology, Pennsylvania State University College of Medicine; Staff Dermatologist, Pennsylvania State Milton S Hershey Medical Center

Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Society for Investigative Dermatology, Association of Professors of Dermatology, North American Hair Research Society

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Carrie L Kovarik, MD Assistant Professor of Dermatology, Dermatopathology, and Infectious Diseases, University of Pennsylvania School of Medicine

Carrie L Kovarik, MD is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Nothing to disclose.

Acknowledgements

Michael G Bryan, MD Staff Dermatologist, Dermatology, Las Vegas Skin and Care Clinics

Disclosure: Nothing to disclose.

Robin L Hornung, MD, MPH Assistant Professor, Division of Dermatology, Department of Pediatrics, University of Washington School of Medicine; Director, Department of Pediatric Dermatology, Children's Hospital and Regional Medical Center, Seattle

Robin L Hornung, MD, MPH is a member of the following medical societies: American Academy of Dermatology, Society for Pediatric Dermatology, and Women's Dermatologic Society

Disclosure: Nothing to disclose.

Lester F Libow, MD Dermatopathologist, South Texas Dermatopathology Laboratory

Lester F Libow, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Texas Medical Assocation

Disclosure: Nothing to disclose.

Jonathan M Olson, MD Fellow, Division of Dermatology, University of Washington Medical Center

Jonathan M Olson, MD is a member of the following medical societies: American Medical Association

Disclosure: Nothing to disclose.

David T Robles, MD, PhD Dermatologist, Chaparral Medical Group

David T Robles, MD, PhD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References
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Thickened, fragmented, hyperkeratotic nails and erythematous periungual skin. Courtesy of Walter Reed Army Medical Center.
Crusted hyperkeratotic plaques on and around the nose. Courtesy of Walter Reed Army Medical Center.
Crusted hyperkeratotic plaques on eyebrow, forehead, and scalp. Courtesy of Walter Reed Army Medical Center.
 
 
 
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