Chronic Mucocutaneous Candidiasis 

  • Author: David T Robles, MD, PhD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Sep 29, 2011
 

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 erThickened, 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]

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Epidemiology

Mortality/Morbidity

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, 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.[4] 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.

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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Contributor Information and Disclosures
Author

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.

Coauthor(s)

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.

Jonathan M Olson, MD  Resident Physician, 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.

Specialty Editor Board

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.

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, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

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, Association of Professors of Dermatology, North American Hair Research Society, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

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

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors, Michael G. Bryan, MD and Lester F. Libow, MD to the development and writing of this article.

References

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  2. Lilic D, Gravenor I, Robson N, Lammas DA, Drysdale P, Calvert JE, et al. Deregulated production of protective cytokines in response to Candida albicans infection in patients with chronic mucocutaneous candidiasis. Infect Immun. Oct 2003;71(10):5690-9. [Medline].

  3. van de Veerdonk FL, Plantinga TS, Hoischen A, et al. STAT1 mutations in autosomal dominant chronic mucocutaneous candidiasis. N Engl J Med. Jul 7 2011;365(1):54-61. [Medline].

  4. Marazzi MG, Bondi E, Giannattasio A, Strozzi M, Savioli C. Intracranial aneurysm associated with chronic mucocutaneous candidiasis. Eur J Pediatr. Apr 19 2007;[Medline].

  5. Herrod HG. Chronic mucocutaneous candidiasis in childhood and complications of non-Candida infection: a report of the Pediatric Immunodeficiency Collaborative Study Group. J Pediatr. Mar 1990;116(3):377-82. [Medline].

  6. Brännström J, Hässler S, Peltonen L, Herrmann B, Winqvist O. Defect internalization and tyrosine kinase activation in Aire deficient antigen presenting cells exposed to Candida albicans antigens. Clin Immunol. Dec 2006;121(3):265-73. [Medline].

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  8. Atkinson TP, Schäffer AA, Grimbacher B, Schroeder HW Jr, Woellner C, Zerbe CS, et al. An immune defect causing dominant chronic mucocutaneous candidiasis and thyroid disease maps to chromosome 2p in a single family. Am J Hum Genet. Oct 2001;69(4):791-803. [Medline].

  9. Patiroglu T, Tahan F. Chronic mucocutaneous candidiasis with agammaglobulinaemia. J Eur Acad Dermatol Venereol. Jul 2007;21(6):833-4. [Medline].

  10. Meager A, Visvalingam K, Peterson P, Möll K, Murumägi A, Krohn K, et al. Anti-interferon autoantibodies in autoimmune polyendocrinopathy syndrome type 1. PLoS Med. Jul 2006;3(7):e289. [Medline].

  11. Masi M, De Vinci C, Baricordi OR. Transfer factor in chronic mucocutaneous candidiasis. Biotherapy. 1996;9(1-3):97-103. [Medline].

  12. Abuzakouk M, Feighery C. Primary immunodeficiency disorders in the Republic of Ireland: first report of the national registry in children and adults. J Clin Immunol. Jan 2005;25(1):73-7. [Medline].

  13. Chiu SJ, Tsao CH, Chen LC, Kao CC, Lue KH, Huang JL. Chronic mucocutaneous candidiasis in a 6-year-old boy. J Microbiol Immunol Infect. Jun 2004;37(3):196-9. [Medline].

  14. Ee HL, Tan HH, Ng SK. Autosomal dominant familial chronic mucocutaneous candidiasis associated with acne rosacea. Ann Acad Med Singapore. Oct 2005;34(9):571-4. [Medline].

  15. Guidelines/Outcome Committee, American Academy of Dermatology. Guidelines of care for superficial mycotic infections of the skin: mucocutaneous candidiasis. J Am Acad Dermatol. Jan 1996;34(1):110-5. [Medline].

  16. Kirkpatrick CH. Chronic mucocutaneous candidiasis. Pediatr Infect Dis J. Feb 2001;20(2):197-206. [Medline].

  17. Mangino M, Salpietro DC, Zuccarello D, Gangemi S, Rigoli L, Merlino MV, et al. A gene for familial isolated chronic nail candidiasis maps to chromosome 11p12-q12.1. Eur J Hum Genet. Jun 2003;11(6):433-6. [Medline].

  18. Perheentupa J. Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy. J Clin Endocrinol Metab. Aug 2006;91(8):2843-50. [Medline].

 
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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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