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Cmc(de Moraes-Vasconcelos, Orii et al. 2001; Kirkpatrick 2001; Lilic and Gravenor 2001;
Palma-Carlos and Palma-Carlos 2001; Tay and Seow 2001; Lilic 2002; Ree, Jennings et al. 2002;
Bhowate and Dubey 2004; Chiu, Tsao et al. 2004; Dixon, Steinbach et al. 2004; Myhre,
Stray-Pedersen et al. 2004; Fazlollahi, Farhoudi et al. 2005; Sathishkumar, Scott et al. 2005; San
Filippo 2006; Patiroglu and Tahan 2007; Rosa, Pasqualotto et al. 2008; Browne and Holland 2010;
Eyerich, Eyerich et al. 2010)

Bhowate, R. and A. Dubey (2004). "Chronic mucocutaneous candidiasis: a case report." J Indian Soc
Pedod Prev Dent 22(1): 21-23.
        Chronic mucocutaneous candidiasis is a immuno deficiency disorder primarily due to T cell
        dysfunction characterized by persistent candidal infection of mucous membrane, skin, scalp
        and nails. Chronic mucous membrane candidiasis has an onset in infancy or childhood; the
        primary affected site is the oral cavity; however, lesions may occur on trunk, hands, feet and
        scalp. This paper describes a 12-year-old girl with candidial infection of the oral mucosa and
        extra oral involvement of fingers, nails, toes and intertragus area.

Browne, S. K. and S. M. Holland (2010). "Anti-cytokine autoantibodies explain some chronic
mucocutaneous candidiasis." Immunol Cell Biol 88(6): 614-615.

Chiu, S. J., C. H. Tsao, et al. (2004). "Chronic mucocutaneous candidiasis in a 6-year-old boy." J
Microbiol Immunol Infect 37(3): 196-199.
        Chronic mucocutaneous candidiasis (CMC) is a complex disorder characterized by persistent
        or recurrent candidal infections of the skin, nails and/or mucous membranes. A familial
        occurrence has been reported in some instances, suggesting a genetic predisposition. CMC has
        also been suggested to be associated with a selective defect in T cell-mediated immunity to
        Candida antigens. Reports of cases in Asians are rare. We report a case of CMC in a
        6-year-old boy with chronic candidal infection since 7 months of age. The patient presented
        with deficient cell-mediated immunity and decreased natural killer cells. This case highlights
        the need for detailed studies for evaluating the T-cell immunity in patients with chronic
        candial infection.

de Moraes-Vasconcelos, D., N. M. Orii, et al. (2001). "Characterization of the cellular immune function
of patients with chronic mucocutaneous candidiasis." Clin Exp Immunol 123(2): 247-253.
        Chronic mucocutaneous candidiasis (CMC) is a rare syndrome characterized by persistent and
        refractory infections of the skin, nails and mucosal tissues by yeasts of the genus Candida.
        Defects in the cellular limb of the immune system are well documented in CMC patients, but
        non-specific immune defects, such as myeloperoxidase deficiency or phagocyte chemotaxis
        disorders, have also been described. Nonetheless, the underlying defect(s) remains poorly
        understood, and further studies are required. We studied eight CMC patients without
        endocrinopathies, who showed (i) low normal proliferative response to phytohaemagglutinin
        (PHA), (ii) partially defective response to pokeweed mitogen (PWM), and (iii) impaired
        response to Candida and PPD antigens. Furthermore, peripheral blood mononuclear cells
        (PBMC) from CMC patients produced lower levels of type-1 cytokines (IL-2 and
         interferon-gamma) in response to Candida antigens, compared with control individuals.
         Conversely, we did not observe an enhancement of IL-4 and IL-10 in the patients, suggesting
         that, even though Th1 cytokines are decreased, the Th2 response is not increased in CMC.
         Nevertheless, the synthesis of these cytokines was normal when induced by PHA. We also
         observed an increased antigen-induced apoptosis in lymphocytes from the patients compared
         with controls, and this applied both to Candida and PPD antigens. Lastly, innate immunity
         defects were investigated. We observed an impairment of natural killer activity against K-562
         target cells in half of the studied patients. These findings corroborate the extensive clinical and
         laboratory variability of CMC, which requires further studies on a larger number of patients to
         be better understood.

Dixon, T. C., W. J. Steinbach, et al. (2004). "Disseminated Candida tropicalis in a patient with chronic
mucocutaneous candidiasis." South Med J 97(8): 788-790.
         Chronic mucocutaneous candidiasis is a heterogeneous group of immunodeficiencies
         associated with persistent candidal infections. Patients with chronic mucocutaneous
         candidiasis are rarely associated with systemic infections caused by other fungi, but almost
         never by Candida. The authors report a case of a 16-year-old with chronic mucocutaneous
         candidiasis who developed a fungemia with Candida tropicalis.

Eyerich, K., S. Eyerich, et al. (2010). "Chronic mucocutaneous candidiasis, from bench to bedside."
Eur J Dermatol 20(3): 260-265.
         Chronic mucocutaneous candidiasis (CMC) defines a heterogeneous group of orphan and
         inherited syndromes characterised by chronic and recurrent infections of the skin and mucosa
         with the yeast Candida. Increasing evidence suggests that this inefficient defence against
         Candida species is reflected by a DC/T cell defect which results in an impaired Th17 and Th1
         immune response and, consecutively, a failed immune instruction of tissue cells. Little is
         known about the incidence and prognosis of CMC. Clinically, the main complications are
         debilitating hands (Candida granuloma) and oesophageal stricture                  with potential
         mal-digestion/-absorption. Furthermore, the chronic infections are likely a risk factor for the
         development of squamous cell carcinoma. Since resistance to anti-mycotic drugs evolves
         rapidly, efficient and flexible therapeutic management is essential for CMC patients.

Fazlollahi, M. R., A. Farhoudi, et al. (2005). "Chronic mucocutaneous candidiasis; report of three cases
with different phenotypes." Iran J Allergy Asthma Immunol 4(1): 39-42.
         Chronic Mucocutaneous Candidiasis (CMCC) refers to a group of immunodeficiencies,
         characterized by persistent or recurrent infections of the skin, nails, and mucus membranes
         caused by candida. A wide range of immunologic abnormality has been reported in CMCC.
         Defects in cellular limb of the immune system, mainly the specific response to antigens of
         candida species, are well documented in CMCC patients. A subgroup of patients is
         predisposed to development of autoimmune endocrinopathies. These patients need repeated
         monitoring of endocrine functions. Immunologic studies are needed to identify the extent of
         immunodeficiency and other abnormalities of immune functions. We report three cases of
         CMCC. These patients show different phenotypes and highlight the need for complete
         evaluation and long term follow-up for accompanying disorders.
Kirkpatrick, C. H. (2001). "Chronic mucocutaneous candidiasis." Pediatr Infect Dis J 20(2): 197-206.
        Chronic mucocutaneous candidiasis should be viewed as a spectrum of disorders in which the
        patients have persistent and/or recurrent candidiasis of the skin, nails and mucous membranes.
        Some of the conditions have genetic predispositions. A common immunologic abnormality is
        failure of the patient's T lymphocytes to produce cytokines that are essential for expression of
        cell-mediated immunity to Candida. Antifungal drugs are effective in clearing the infections,
        and treatments that restore cellular immunity have produced long term remissions.

Lilic, D. (2002). "New perspectives on the immunology of chronic mucocutaneous candidiasis." Curr
Opin Infect Dis 15(2): 143-147.
        Chronic mucocutaneous candidiasis is a primary immune deficiency presenting as an inability
        to clear fungal infections and consequently as persisting and recurring infections of the skin
        and mucous membranes with yeasts, mostly Candida albicans. Chronic mucocutaneous
        candidiasis is a heterogeneous clinical syndrome which usually presents in childhood and can
        have an autosomal recessive, dominant or sporadic mode of inheritance. Most chronic
        mucocutaneous candidiasis patients also develop accompanying endocrine and inflammatory
        disorders that suggest an underlying deregulation of the immune system. 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. The defect underlying altered cytokine production remains unknown but
        studies are in progress addressing the putative role of dendritic cells and pattern recognition
        receptors in directing cytokine responses. These novel insights into immune mechanisms
        responsible for protection against Candida spp. are opening new possibilities of
        immunomodulation and vaccination that could prove beneficial in the management of chronic
        mucocutaneous candidiasis.

Lilic, D. and I. Gravenor (2001). "Immunology of chronic mucocutaneous candidiasis." J Clin Pathol
54(2): 81-83.

Myhre, A. G., A. Stray-Pedersen, et al. (2004). "Chronic mucocutaneous candidiasis and primary
hypothyroidism in two families." Eur J Pediatr 163(10): 604-611.
        We describe the clinical and immunological features of two families with chronic
        mucocutaneous candidiasis (CMC) and primary hypothyroidism. Family A includes three
        siblings with both candidiasis and hypothyroidism and four individuals with hypothyroidism
        only. Family B includes four members with candidiasis, of whom one (a male child) also had
        hypothyroidism. All individuals affected with CMC had suffered from oral candidiasis and
        onychomycosis since infancy. Facial seborrhoic dermatitis, general folliculitis and scaling
        blepharitis were main manifestations. Hypothyroidism became evident during childhood. No
        thyroid antibodies were present in the affected siblings in family A, while the male in family B
        with hypothyroidism had antibodies against thyroid peroxidase at diagnosis. Immunological
        evaluation revealed intra-individual variations in serum immunoglobulin levels, lymphocyte
        subsets and proliferative responses, but there were no consistent abnormalities. Vaccine
        responses were normal. AIRE gene region microsatellite markers did not segregate with
        disease nor were autoantibodies typical for autoimmune polyendocrine syndrome type 1
        detected in the families. Conclusion: The link between hypothyroidism and chronic
        mucocutaneous candidiasis remains to be identified.

Palma-Carlos, A. G. and M. L. Palma-Carlos (2001). "Chronic mucocutaneous candidiasis revisited."
Allerg Immunol (Paris) 33(6): 229-232.
        A study of 26 female patients with recurrent candidial infections have been studied. Delayed
        hypersensitivity to Candida antigens was observed only in 17 of the patients. 12 patients had
        also chronic urticaria. A defect in NK cells was found in 18 out of 23 cases (78%). Treatment
        with Candida vaccines had obtained good clinical results in 11 of the first 13 patients treated.

Patiroglu, T. and F. Tahan (2007). "Chronic mucocutaneous candidiasis with agammaglobulinaemia." J
Eur Acad Dermatol Venereol 21(6): 833-834.

Ree, T., P. R. Jennings, et al. (2002). "Chronic mucocutaneous candidiasis." JAAPA 15(4): 67-70.

Rosa, D. D., A. C. Pasqualotto, et al. (2008). "Chronic mucocutaneous candidiasis and oesophageal
cancer." Med Mycol 46(1): 85-91.
        Chronic mucocutaneous candidiasis (CMC) is often accompanied by endocrine or
        inflammatory disorders. The association of CMC with squamous cell carcinoma of the oral
        cavity    or   oesophagus     have    been    described     in   patients   with    autoimmune
        polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED). We describe three cases of
        CMC and oesophageal cancer without the APECED syndrome. The first case refers to a
        41-year-old man with Candida paronychia and oral infection and selective IgA deficiency
        since childhood, who later developed an oesophageal cancer. The second case is a 30-year-old
        man who presented CMC features at the age of 2 together with selective IgA deficiency. Later
        on he was diagnosed with an oesophageal squamous cell carcinoma. His mother, the third case
        reported, had oral thrush since childhood and at the age of 29 she presented with an
        oesophageal squamous cell carcinoma. The three patients reported died due to oesophageal
        cancer. This is the first case report describing the development of oesophageal cancer in
        patients with CMC without the APECED syndrome. Patients with CMC need close follow-up
        with good oral hygiene and aggressive treatment of oral and oesophageal candidiasis. Routine
        endoscopic screening for patients with CMC that develop symptoms of oesophageal
        candidiasis and for patients with CMC with a family history of oesophageal cancer is
        suggested. Avoidance of additional risk factors for oral and oesophageal cancer like cigarette
        smoking and excessive alcohol consumption are also warranted.

San Filippo, J. (2006). "Chronic mucocutaneous candidiasis associated with malignant thymoma and
systemic lupus erythematosus with hypergammaglobulinemia: a case report and literature review."
Cutis 78(1): 57-60.
        Chronic mucocutaneous candidiasis (CMC) is characterized by recurrent candidal infections
        of the mucous membranes, nails, and skin. Systemic involvement is rare. CMC in adults with
        coexistent thymoma, benign or malignant, is well-known and is often associated with
        hypogammaglobulinemia. There is an unusually high frequency of thymoma and systemic
        lupus erythematosus (SLE). I present a case of a patient with a history of malignant thymoma,
        SLE, and hypergammaglobulinemia who was found to have CMC. Discussion of the
        relationship of these findings is presented.

Sathishkumar, T., J. X. Scott, et al. (2005). "Chronic mucocutaneous candidiasis in a child." Indian J
Dermatol Venereol Leprol 71(6): 432-433.

Tay, Y. K. and C. S. Seow (2001). "What syndrome is this? Chronic mucocutaneous candidiasis."
Pediatr Dermatol 18(4): 353-355.

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