CMC 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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