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1/17. Autoimmune hemolytic anemia in a patient with autosomal dominant chronic mucocutaneous candidiasis.

    Chronic mucocutaneous candidiasis is a heterogeneous immunodeficiency syndrome characterized by recurrent candidal infections of the skin, nails, and mucous membranes. The syndrome can be associated with autoimmune conditions, especially endocrine disorders. Typically, inheritance is autosomal recessive, and abnormal T-cell-mediated immunity is thought to be the underlying deficit. We describe a 27-year-old man with chronic mucocutaneous candidiasis inherited in an autosomal dominant fashion, in whom both lymphocyte blastogenesis and delayed-type skin reactivity to Candida antigens were normal. Notable features of the case include autoimmune hemolytic anemia, probable hypoparathyroidism, and hypogonadal hypogonadism.
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keywords = candida
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2/17. Autoimmune polyglandular syndrome (APS) type 1 and candida onychomycosis.

    A 21-year-old female presented at age 2 years with a chronic mucocutaneous candidiasis and at age 3 alopecia totalis. Later, chronic hypoparathyroidism and autoimmune adrenal insufficiency appeared. In addition, malabsorption syndrome and signs of pernicious anaemia occurred. The onychomycosis totally improved under systemic treatment with fluconazole (Diflucan), endocrine and organ failure with replacement therapy. The autoimmune polyglandular syndrome (APS 1) is a rare autosomal recessive inherited disease. Chronic mucocutaneous candidiasis (CMC) generally presents very early in life and is the most frequent of the three main diseases of APS type 1 (chronic hypoparathyroidism, autoimmune Addison's disease). It can be considered as a precocious marker of APS type 1. Consequently, all patients affected by isolated CMC, especially children, should be evaluated and carefully followed up by immunological, biochemical, and clinical tests to recognize signs and symptoms of imminent or ongoing endocrine glandular failure.
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ranking = 4
keywords = candida
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3/17. Chronic mucocutaneous candidiasis in a 6-year-old boy.

    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.
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ranking = 2
keywords = candida
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4/17. Chronic mucocutaneous candidiasis: a case report.

    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.
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keywords = candida
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5/17. Disseminated candida tropicalis in a patient with chronic mucocutaneous candidiasis.

    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.
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keywords = candida
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6/17. Chronic mucocutaneous candidiasis associated with malignant thymoma and systemic lupus erythematosus with hypergammaglobulinemia: a case report and literature review.

    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.
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keywords = candida
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7/17. Transient abrogation of immunosuppression in a patient with chronic mucocutaneous candidiasis following vaccination with candida albicans.

    A patient with long-standing chronic mucocutaneous candidiasis had reversed T helper/suppressor (TH/TS) cell ratios, hypergammaglobulinaemia E and serum inhibitors of lymphocyte transformation to mitogens and candida antigens. Following vaccination with whole heat-killed yeasts of candida albicans, temporary clinical improvement coincided with the return of the TH/TS cell ratio to normal, reduction in concentration of IgE and reduced serum inhibitory activity to concanavalin a and candida antigens. These changes were not permanent and 6 months after vaccination all indices had reverted to their pretreatment values. The production of antibody to a 47 kDa antigen of C. albicans has been shown to coincide with recovery from systemic disease. High concentrations of this antibody were demonstrated initially in the patient's serum and were unaffected by vaccination. If the 47 kDa antibody is protective, the results of this study suggest that separate immune responses may protect against mucocutaneous and systemic candidiasis and that defective immune responses against mucocutaneous disease need not imply lack of protection against systemic spread.
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ranking = 2
keywords = candida
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8/17. Chronic mucocutaneous candidiasis with iga deficiency in a two-year-old African girl who responded well to ketoconazole.

    The rare disorder of chronic mucocutaneous candidiasis is described in a two-year-old African girl who had a moderate lymphopenia due to fewer T and B cells, impaired lymphocyte transformation to PHA and candida antigen, decreased leucocyte migration inhibition to candida antigen and a defective cutaneous delayed hypersensitivity reaction to candida antigen and dinitrochlorobenzene. The patient's serum had a marginal inhibitory effect on the transformation of normal lymphocytes to candida antigen and no effect on PHA stimulation of these cells, as measured by stimulation index. In addition, IgA was deficient in her serum. No associated endocrinopathy was detected and she had a mild iron deficiency anaemia. There was a rapid and excellent response to ketoconazole with all lesions clearing within two months of treatment.
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ranking = 4
keywords = candida
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9/17. Late-onset chronic mucocutaneous and ocular candidiasis and malignant thymoma.

    Late-onset chronic mucocutaneous candidiasis (CMCC) is part of a spectrum of candidal infections. It usually develops during or after the fourth decade of life and is often associated with an underlying neoplasm, especially thymoma. A 56-year-old man with late-onset CMCC and thymoma had several unusual features. His malignant thymoma seemed to be more widespread than the thymomas of previously described patients. Ocular infection was present in addition to extensive involvement of the skin, nails, and oral mucosa. To our knowledge, this is the first report of ocular involvement in this syndrome. We also believe that this is the first patient with thymoma-associated late-onset CMCC to be treated effectively with ketoconazole.
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ranking = 1
keywords = candida
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10/17. ketoconazole treatment of nail infection in chronic mucocutaneous candidiasis.

    Chronic mucocutaneous candidiasis is an immunodeficiency disorder which has significant morbidity due to mucous membrane, skin and nail infection. Attempts at reconstitution of immunological abnormalities have had only limited success. ketoconazole is a newly available oral antifungal agent with activity against candida species. Prolonged administration of ketoconazole to four children with chronic mucocutaneous candidiasis caused marked improvement at infected sites. Resolution of nail infections took up to 12 months.
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ranking = 1
keywords = candida
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