Chronic Mucocutaneous Candidiasis Workup

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

Laboratory Studies

  • When a patient presents with cutaneous manifestations of CMC, the following studies should be performed:
    • Scrapings from the infected site are suspended in 10-20% KOH and microscopically examined. The presence of yeast cells and pseudohyphae confirms the diagnosis. Fungal stains, such as chlorazol black E stain or Parker blue-black ink, may be added to highlight the organism.
    • Candidal organisms grow well on several culture media. They grow as yeasts on Sabouraud agar with chloramphenicol and cycloheximide. C albicans also grows on dermatophyte test medium but does not demonstrate the red color change characteristic of dermatophytes.
    • Screening laboratory tests for a CMC-associated endocrine dysfunction include blood glucose or glycosylated hemoglobin testing, thyroid function tests, liver functions tests, serum electrolyte evaluation, corticotropin testing, and serum cortisol values. Consider a complete blood cell count, to screen for leukopenia, and an HIV test. Other endocrine screening tests that may be considered include follicle-stimulating hormone, luteinizing hormone, prolactin, testosterone, parathyroid-stimulating hormone, calcium, phosphate, magnesium, and short synacthen test. Perform baseline and yearly follow-up tests to screen for associated endocrinopathy.
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Other Tests

  • Results from cellular immunity tests, such as the prick-test with Candida antigens, may be negative. In vitro lymphocyte proliferation is usually reduced for C albicans extracts.
  • Immunoglobulin G subclass deficiencies have been reported in some patients with CMC, who have a predisposition toward respiratory tract infections. Isolated immunoglobulin A and immunoglobulin M deficiencies have also been reported, in addition to a single case of complete agammaglobulinemia.[9]
  • In patients with other recurrent infections, immune studies should be considered.
  • If the clinical suspicion for APECED is high, genetic analysis of the AIRE gene can be confirmatory.[6, 7]
  • Recently, anti-interferon-1 antibodies were found to be highly specific for APECED and to precede the appearance of CMC, suggesting an important new diagnostic test.[10]
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Procedures

  • A skin biopsy is rarely needed to make a diagnosis of Candida infection, but it may be performed to rule out the possibility of superinfection of a primary dermatosis. A periodic acid-Schiff stain can confirm the presence of pseudohyphae. Nutritional deficiencies with cutaneous manifestations should also be considered.
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Histologic Findings

Routine hematoxylin and eosin–stained sections of superficial candidiasis lesions reveal subcorneal pustules. Granulomatous lesions of CMC show hyperkeratosis and parakeratosis, with a dense mixed dermal infiltrate containing lymphocytes and plasma cells. Periodic acid-Schiff or silver stains of skin biopsy specimens can help identify organisms in the stratum corneum and dermis.

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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

  1. Lilic D. New perspectives on the immunology of chronic mucocutaneous candidiasis. Curr Opin Infect Dis. Apr 2002;15(2):143-7. [Medline].

  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].

  7. Collins SM, Dominguez M, Ilmarinen T, Costigan C, Irvine AD. Dermatological manifestations of autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome. Br J Dermatol. Jun 2006;154(6):1088-93. [Medline].

  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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