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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Chronic mucocutaneous candidiasis (CMCC) is a clinical syndrome characterized by persistent and recurrent Candida albicans infections of the mucous membranes and skin often associated with immunodeficiency. In order to gain insight into the frequency and severity of non-candidal infections in children with CMCC, four patients with CMCC are described in detail and 60 previously reported cases are reviewed. Fifty percent of children with CMCC had significant infections with other fungi, bacteria and viruses. Recurrent bacterial pneumonias and bronchiectasis were a major cause of morbidity and mortality. In addition, there were a large number of infections, in both the lung and other sites, due to opportunistic organisms. Thus the clincial syndrome of CMCC includes not only mucocutaneous candidiasis, endocrine failure and autoimmune phenomena, but patients with CMCC also show a remarkable susceptibility to non-candidal infections. These non-candidal infections represent a serious cause of morbidity for patients with CMCC.
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PMID:Non-candidal infections in children with chronic mucocutaneous candidiasis. 45 1

The authors report the case of a child who, at the age of 18 months showed signs of hypoparathyroidism together with gastrointestinal, then buccal, then ungual candidiasis. Acute adrenal failure occurred when he was 5 1/2 years' old. At the age of 10, the patient developed alopecia areata and interstitial keratitis. Immunological investigations yielded normal results, except that serum was weakly positive for anti-adrenal antibodies at 1/10th. The mucosal and ungual candidiasis infection was cured by ketoconazole, and the various endocrine abnormalities were corrected with the appropriate replacement therapies. This case prompted the authors to review the candidiasis/"polyglandular autoimmune disease" association. Whitaker's triad consists of candidiasis, hypoparathyroidism and chronic renal failure, 2 or these 3 elements being sufficient to make the diagnosis. Numerous other associations have been described; they are presented here in table form in descending order of frequency, with candidiasis/hypoparathyroidism coming on top of the list (70 p. 100). The fairly constant chronological order in which these different pathologies appear is one of the peculiarities of the syndrome: candidiasis often precedes hypoparathyroidism and adrenal insufficiency. Alopecia areata does not seem to be frequent, but its true incidence is difficult to quantify since lesions of the scalp and/or skin appendages are poorly documented in the literature. Alopecia and keratopathy seem to be of autoimmune origin. Mucocutaneous candidiasis too is specific, the mucosae and nails being constantly involved. This type of candidiasis does not exist in other forms of hypoparathyroidism. Chronic mucocutaneous candidiasis is found in many different diseases and is due to immunodeficiency against Candida spp.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Autoimmune polyendocrinopathy and chronic mucocutaneous candidiasis]. 266 Jul 6

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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PMID:Ketoconazole treatment of nail infection in chronic mucocutaneous candidiasis. 631 90

The immune system contains multiple components that provide protection against specific groups of microorganisms. The degree of specialization of function is clearly illustrated in patients with immunodeficiency syndromes in whom the nature of the defect determines the sites of the infections and the susceptibility to certain organisms. Chronic mucocutaneous candidiasis is an especially dramatic illustration of the role of the T-lymphocyte system in defense against opportunistic fungal infections, especially of the skin and mucous membranes. There is considerable heterogeneity of the immune abnormalities in patients with chronic mucocutaneous candidiasis. The most consistent defects involve subnormal production of lymphokines by T-cells in response to Candida antigens. However, some patients have more global defects and are unresponsive to all antigens and may have reduced responsiveness to mitogens as well. Successful therapy of this infection requires a combination of treatments, including such antifungal drugs as clotrimazole, ketoconazole, or amphotericin B and correction of the underlying immune defect with such agents as transfer factor.
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PMID:Host factors in defense against fungal infections. 638 23

Chronic mucocutaneous candidiasis is a rare cellular immunodeficiency disease, characterized by persistent infections of the mucous membranes, nails, skin and scalp. Candidal granuloma is an uncommon form of mucocutaneous candidiasis of early onset which may ne associated with hypothyroidism. It is difficult to manage as so far no truly effective treatment has been found. We present a 13-year old boy with candidal granuloma associated with primary hypothyroidism who had a favorable response to treatment with thyroid hormone and miconazole. The fungicide was given in tablet form, a single dose per day and patient kept it in his mouth and in contact with his lips for 15 minutes. One and a half year later clinical evolution is good and no side effects related to the miconazole have been observed.
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PMID:[Candidiasic granuloma associated to hypothyroidism (author's transl)]. 732 44

Chronic mucocutaneous candidiasis is an immunodeficiency disease characterized by T-cell dysregulation and chronic superficial candidal infections. We report on three patients with chronic mucocutaneous candidiasis who developed autoantibodies to erythrocytes. Our first patient, a 19-year-old female, developed autoimmune hemolytic anemia (AIHA) that required multiple courses of treatment, including corticosteroids, intravenous immunoglobulin, and danazol. During the last exacerbation of AIHA, intensive treatment with corticosteroids and intravenous immunoglobulin failed and yet the patient responded to plasmapheresis. Our second patient, a 21-year-old male, developed AIHA which responded to oral corticosteroid therapy. Our third patient, a 6-year-old female without evidence of hemolysis, was found to have erythrocyte autoantibodies on routine screening. These three patients had positive direct antiglobulin tests, and the first patient had both immunoglobulin G (IgG) and IgM erythrocyte autoantibodies, while the remaining two patients had only IgG autoantibody. This is the first report of the association of AIHA with chronic mucocutaneous candidiasis. We suggest that all patients with chronic mucocutaneous candidiasis be screened periodically for erythrocyte autoantibodies. Plasmapheresis, a safe ancillary procedure in the management of AIHA, may be life-saving in some cases. The occurrence of erythrocyte autoantibodies in mucocutaneous candidiasis may be related to immunoregulatory disorders in this disease.
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PMID:Autoimmune hemolytic anemia in chronic mucocutaneous candidiasis. 749 19

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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PMID:Autoimmune hemolytic anemia in a patient with autosomal dominant chronic mucocutaneous candidiasis. 1094 43

Chronic mucocutaneous candidiasis (CMC) is a primary immunodeficiency disease clinically characterized by Candida infection of the skin, mucous membranes, or nails that is refractory to traditional treatment. We present a typical case of a 13-year-old boy with an onset of illness at 1 month of age in the form of oral thrush. At age 2-3 years the patient began to have external otitis caused by Candida albicans and recurrent upper respiratory tract infections. Analytical studies detected iron deficiency and circulating antigliadin antibodies. Immunologic findings excluded other possible immunodeficiencies. Significant clinical improvement was produced by therapy with orally administered fluconazole. The significance of antigliadin antibodies is discussed.
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PMID:Antigliadin antibodies associated with chronic mucocutaneous candidiasis. 1238 98

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.
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PMID:Chronic mucocutaneous candidiasis; report of three cases with different phenotypes. 1730 22

Chronic mucocutaneous candidiasis (CMC) is a rare disease associated with immunodeficiency and characterized by persistent and refractory infections of the skin, appendages and mucous membranes caused by members of the genus Candida. Several different disorders are classified under this common denominator, including chronic and recurrent mucocutaneous infections due to Candida spp., which are sometimes linked to autoimmune endocrinopathies. These fungal infections are usually confined to the mucocutaneous surface, with little propensity for systemic disease or septicemia. We describe a patient with CMC who had an esophageal candidiasis refractory to treatment for decades and who developed an epidermoid esophageal cancer. No risk factors such as familiar susceptibility, smoking, alcohol drinking, or living in an endemic area were verified. This case report suggests the participation of nitrosamine compounds produced by chronic Candida infections as a risk factor for esophageal cancer in a patient with autosomal-dominant chronic mucocutaneous candidiasis.
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PMID:Esophageal cancer associated with chronic mucocutaneous candidiasis. Could chronic candidiasis lead to esophageal cancer? 1879 15


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