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Chronic Mucocutaneous Candidiasis - Causes, Symptoms and Treatment

Definition:

Chronic mucocutaneous candidiasis is a form of candidiasis (moniliasis) that usually develops in very small children of less than 1 year age, but may sometimes occur occasionally in the late 20s. Affecting males and females, it's characterized by repeated infection with Candida albicans that may result from an inherited defect in cell-mediated (T-cell) immunity. In some patients, an autoimmune response affecting the endocrine system may induce various endocrinopathies.

Despite chronic candidiasis, these patients seldom die of systemic infection. Instead, they usually die of hepatic or endocrine failure. The prognosis for chronic mucocutaneous candidiasis depends on the severity of the associated endocrinopathy. Patients with associated endocrinopathy seldom live beyond their 30s.

Causes

No characteristic immunologic defects have been identified in this infection, but many patients have a diminished response to various antigens or to Candida alone. In some patients, anergy may result from deficient migration inhibition factor, a mediator normally produced by lymphocytes.

Signs and Symptoms

Chronic candidal infections can affect the skin, mucous membranes, nails, and vagina, usually causing large, circular lesions. These infections seldom produce systemic symptoms, but in late stages may be associated with recurrent respiratory tract infections. Other associated conditions include severe viral infections that may precede the onset of endocrinopathy and, sometimes, hepatitis. Involvement of the mouth, nose, and palate may cause speech and eating difficulties.

Symptoms of endocrinopathy are peculiar to the organ involved. Tetany and hypocalcemia are most common and are associated with hypoparathyroidism. Addison's disease, hypothyroidism, diabetes, and pernicious anemia are also connected with chronic mucocutaneous candidiasis. Psychiatric disorders are likely be­cause of disfigurement and multiple endocrine aberrations.

Diagnosis

Laboratory findings usually show a normal circulating T-cell count, although it may be decreased. Skin tests don't usually show delayed hypersensitivity to Candida, even during the infectious stage. Migration inhibiting factor that indicates the presence of activated T cells may not respond to Candida.

Nonimmunologic abnormalities resulting from endocrinopathy may include hypocalcemia, abnormal hepatic function studies, hyperglycemia, iron deficiency, and abnormal vitamin B12 absorption (pernicious anemia). Diagnosis must rule out other immunodeficiency disorders associated with chronic Candida infection, especially DiGeorge syndrome, ataxiatelangiectasia, and severe combined immunodeficiency disease, all of which produce severe immunologic defects. After diagnosis, the patient needs evaluation of adrenal, pituitary, thyroid, gonadal, pancreatic, and parathyroid function as well as careful follow-up. The disease is progressive, and most patients eventually develop endocrinopathy.

Treatment for Chronic Mucocutaneous Candidiasis

Treatment aims to control infection but is not always successful. Topical anti fungal agents are often ineffective against chronic mucocutaneous candidiasis. Miconazole and nystatin are sometimes useful but ultimately fail to control this infection.

Systemic infections may not be fatal, but they're serious enough to warrant vigorous treatment. Oral ketoconazole and injected thymosin and levamisole have had some positive effect. Oral or I. M. iron replacement may also be necessary. Treatment may also include plastic surgery, when possible, and counseling to help patients cope with their disfigurement

Prevention Tips for Controlling Chronic Mucocutaneous Candidiasis

Teach the patient about the progressive manifestations of the disease, and emphasize the importance of seeing an endocrinologist for regular checkups.

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