Chronic Mucocutaneous Candidiasis

A clinical syndrome characterized by development, usually in infancy or childhood, of a chronic, often widespread candidiasis of skin, nails, and mucous membranes. It may be secondary to one of the immunodeficiency syndromes, inherited as an autosomal recessive trait, or associated with defects in cell-mediated immunity, endocrine disorders, dental stomatitis, or malignancy.

Also Known As:

Networked: 195 relevant articles (12 outcomes, 8 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Infection
2.	Candidiasis (Moniliasis)
3.	Acquired Immunodeficiency Syndrome (AIDS)
4.	IgA Deficiency
5.	Histoplasmosis

Experts

1.	Casanova, Jean-Laurent: 12 articles (04/2014 - 02/2010)
2.	Puel, Anne: 9 articles (04/2014 - 02/2010)
3.	Lilic, Desa: 8 articles (10/2015 - 04/2002)
4.	Cypowyj, Sophie: 8 articles (04/2014 - 04/2011)
5.	Picard, Capucine: 8 articles (12/2013 - 02/2010)
6.	Abel, Laurent: 6 articles (10/2013 - 02/2010)
7.	Maródi, László: 5 articles (02/2014 - 08/2011)
8.	Bustamante, Jacinta: 5 articles (12/2013 - 02/2010)
9.	Al-Muhsen, Saleh: 5 articles (03/2013 - 02/2010)
10.	Netea, Mihai G: 4 articles (10/2015 - 12/2010)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Chronic Mucocutaneous Candidiasis:

1.	Ketoconazole (Nizoral)FDA LinkGeneric 09/01/1983 - "Prolonged administration of ketoconazole to four children with chronic mucocutaneous candidiasis caused marked improvement at infected sites. " 11/01/1982 - "We report the clinical and laboratory findings in two children with chronic mucocutaneous candidiasis (CMC) treated successfully with intermittent long-term ketoconazole therapy. " 12/01/1980 - "Oral ketoconazole is an effective treatment for chronic mucocutaneous candidiasis." 01/24/1983 - "It is possible to maintain some patients with chronic mucocutaneous candidiasis in remission without using prophylactic ketoconazole, although relapses may occur. " 01/01/1987 - "Immunological studies in chronic mucocutaneous candidiasis before and after ketoconazole treatment."
2.	Interleukin-17 (Interleukin 27)IBA 01/01/2015 - "During recent years, inborn errors of human IL-17 immunity have been demonstrated to underlie primary immunodeficiencies with chronic mucocutaneous candidiasis (CMC). " 04/01/2014 - "IL-17 T cells' defective differentiation in vitro despite normal range ex vivo in chronic mucocutaneous candidiasis due to STAT1 mutation." 03/01/2014 - "Interleukin-17 (IL-17) deficiency in mice or humans leads to chronic mucocutaneous candidiasis, but the specific downstream mechanisms of IL-17-mediated host defense remain unclear. " 10/17/2013 - "Patients with inborn errors of interleukin-17F (IL-17F) or IL-17RA display chronic mucocutaneous candidiasis (CMC). " 10/17/2013 - "An ACT1 mutation selectively abolishes interleukin-17 responses in humans with chronic mucocutaneous candidiasis."
3.	CytokinesIBA 06/01/2012 - "Thorough analyses of cohorts of patients with unusual susceptibility to chronic mucocutaneous candidiasis resulting from T(H)17 deficiency have confirmed the role of T(H)17 cells and T(H)17 cytokines in human host defense against Candida species and have provided valuable insights into the complex process of T(H)17 cell development." 02/01/2012 - "In patients with chronic mucocutaneous candidiasis, gain-of-function STAT1 mutations shift the cellular response toward T(H)17 cell-inhibiting cytokines. " 11/01/1996 - "Production of T-helper cell subsets and cytokines by lymphocytes from patients with chronic mucocutaneous candidiasis." 08/01/2015 - "Chronic mucocutaneous candidiasis constitutes a heterogeneous group of syndromes, characterized by non-invasive infection of the skin, nails and mucosal membranes by the fungus Candida spp. Although symptoms are heterogeneous, in all cases there is a reduction in protective cytokines, favouring the development of disease. " 02/15/2010 - "Chronic mucocutaneous candidiasis in APECED or thymoma patients correlates with autoimmunity to Th17-associated cytokines."
4.	Transfer Factor (Factor, Transfer)IBA 09/01/1976 - "Clinical and immunological improvement in a patient with chronic mucocutaneous candidiasis treated with transfer factor." 01/01/1980 - "Transfer factor in the treatment of chronic mucocutaneous candidiasis: a controlled study." 01/01/1996 - "Transfer factor in chronic mucocutaneous candidiasis." 01/01/1980 - "A controlled cross-over study with transfer factor was carried out on 7 patients suffering from chronic mucocutaneous candidiasis. " 05/01/1976 - "The effect of transfer factor on neutrophil function in chronic mucocutaneous candidiasis."
5.	Interleukin-10 (Interleukin 10)IBA 04/01/2002 - "It has long been recognized that protection from mucocutaneous candidiasis relies on cell-mediated immunity and studies on animal models have highlighted the essential role of type 1 cytokines in protection against Candida spp. Recent data in patients with chronic mucocutaneous candidiasis have documented altered patterns of cytokine production in response to Candida spp. with decreased production of some but not all type 1 cytokines and increased levels of interleukin-10. "
6.	Itraconazole (Sporanox)FDA LinkGeneric 03/01/1997 - "We report on two children affected by chronic mucocutaneous candidiasis involving the mouth and all the nails who were successfully treated with itraconazole at 200 mg/day for 2 months. " 03/01/1997 - "Itraconazole in the treatment of two young brothers with chronic mucocutaneous candidiasis." 12/01/1989 - "Use of itraconazole in a patient with chronic mucocutaneous candidiasis." 03/01/1989 - "Preliminary findings also indicate that itraconazole may hold promise for the prophylaxis of opportunistic fungal infections in patients at risk, for example women with chronic recurrent vaginal candidiasis, immunodeficient patients with chronic mucocutaneous candidiasis, AIDS patients and patients receiving immunosuppressant drugs. "
7.	Cimetidine (Biomet)FDA LinkGeneric 02/01/1980 - "Four adult patients with chronic mucocutaneous candidiasis were studied to establish a possible role for cimetidine as an immunomodulator. " 02/01/1980 - "Cimetidine as an immunomodulator: chronic mucocutaneous candidiasis as a model." 10/01/1979 - "Cimetidine in chronic mucocutaneous candidiasis." 04/01/1996 - "The authors evaluated the clinical efficacy of a treatment with cimetidine and zinc sulphate in a patient with chronic mucocutaneous candidiasis. " 04/01/1996 - "Case report: successful treatment with cimetidine and zinc sulphate in chronic mucocutaneous candidiasis."
8.	Granulocyte Colony-Stimulating Factor (G-CSF)IBA 09/01/2015 - "Immunotherapy with G-CSF in patients with chronic mucocutaneous candidiasis." 09/01/2013 - "Continuous G-CSF therapy for isolated chronic mucocutaneous candidiasis: complete clinical remission with restoration of IL-17 secretion."
9.	Systemic candidiasisIBA 08/01/2013 - " newborn eczematous eruption, which occurs mainly in the signal-transducer-and-activator-of-transcription-3 (STAT3) variant, helps to differentiate STAT3 from Dedicator of Cytokinesis 8-related Hyper-IgE-syndrome (HIES).Impaired T helper 17 cell (TH17) immunity [HIES and defects of autoimmune regulator element (AIRE), STAT-1, and interleukin17 receptor(IL17(R))] may give rise to localized chronic mucocutaneous candidiasis, whereas a defective innate immune system predisposes to systemic candidiasis [congenital neutropenia, neutrophil dysfunction, and caspase recruitment domain 9 (CARD9) deficiency].Noninfectious granulomas may be the presenting symptom in innate immunity defects [such as chronic granulomatous disease (CGD) or in predominantly humoral immunodeficiencies such as common variable immunodeficiency], as well as ataxia teleangiectasia or rare recombination-activating gene-deficient cases. " 01/24/1983 - "Excellent results were obtained in six cases of chronic mucocutaneous candidiasis, with restoration of immunologic disturbances; 23 cases of systemic candidiasis, including new aspects of heroin addicts with cutaneous, ocular, or osteoarticular manifestations; eight cases of histoplasmosis, five due to Histoplasma capsulatum and three to Histoplasma duboisii, with cure in seven and remission in one; one case of African blastomycosis (Blastomyces dermatitidis); three cases of mycetoma, two due to Monosporium apiospermum, one due to a dematiacious fungus; three cases of entomophthoromycosis with cure; one case of fungal arthritis, due to new hyphomycete similar to M. "
10.	ZincIBA 04/01/1996 - "The authors evaluated the clinical efficacy of a treatment with cimetidine and zinc sulphate in a patient with chronic mucocutaneous candidiasis. " 04/01/1996 - "Case report: successful treatment with cimetidine and zinc sulphate in chronic mucocutaneous candidiasis."

Therapies and Procedures

1.	Immunotherapy 03/01/1979 - "Treatment of chronic mucocutaneous candidiasis has included drugs, immunotherapy, and replacement of nutritional and endocrine deficiency. " 11/01/1977 - "Combination immunotherapy in chronic mucocutaneous candidiasis. " 09/01/2015 - "Immunotherapy with G-CSF in patients with chronic mucocutaneous candidiasis."
2.	Transplantation (Transplant Recipients) 10/23/1971 - "Thymic transplantation in a case of chronic mucocutaneous candidiasis." 07/01/1978 - "Other antifungals and immune system-stimulating modalities (transfer factor, thymosin, thymus epithelial cell transplantation, and levamisol) may be useful for chronic mucocutaneous candidiasis in some settings and deserve further evaluation."
3.	Bone Marrow Transplantation (Transplantation, Bone Marrow) 10/01/1996 - "Successful allogeneic bone marrow transplantation in severe chronic mucocutaneous candidiasis syndrome." 10/01/1996 - "We report the successful HLA-identical sibling bone marrow transplantation (BMT) of a 12-year-old boy with chronic mucocutaneous candidiasis (CMC) syndrome who also had Coomb's positive haemolytic anaemia and recurrent bronchopneumonia with resulting bronchiectasis. "
4.	Vertical Dimension (Occlusion Vertical Dimension) 10/30/1984 - "Other forms that affect the mouth include acute atrophic candidiasis associated with oral antibiotic therapy, chronic atrophic candidiasis attributable to the wearing of full upper dentures, candidal cheilosis ascribable to perlèche or decreased vertical dimension in the lower third of the face, chronic mucocutaneous candidiasis emanating from a T-lymphocyte deficiency, and candidal leukoplakia. "
5.	Radiotherapy 05/01/1980 - "Despite disappearance of the inhibitor after apparently curative radiotherapy, suggesting the tumour was the origin, the chronic mucocutaneous candidiasis persists. "