Paracoccidioidomycosis (PCM) is a chronic infectious tropical disease caused by the fungus Paracoccidioides brasiliensis. The initial infection usually occurs in the lungs, but may also spread to the skin, mucous membranes, and other parts of the body. Specialized cells that line the walls of blood and lymphatic vessels and dispose of cellular waste (reticuloendothelial system) may also be affected by paracoccidioidomycosis. If the patient does not receive treatment, life-threatening complications can occur. Most cases of this disease occur in South and Central America.
The symptoms of paracoccidioidomycosis generally occur from several weeks or months to years after the initial exposure to the fungus. The symptoms vary according to which areas of the body are infected.
The symptoms of pulmonary paracoccidioidomycosis, in which the lungs are affected, may include cough, difficulty breathing (dyspnea), fatigue, and/or chest pain. Adults with this form of the disorder may also have fibrous and degenerative changes in the lungs that cause the progressive loss of lung function (emphysema). In some people, the symptoms of paracoccidioidomycosis progress to a condition known as cor pulmonale. Heart disease occurs in this condition because of abnormally high blood pressure within the vessels that move blood away from the lungs and toward the heart.
In mucocutaneous paracoccidioidomycosis, ulcers (granulomatous lesions) appear on the mucous membranes, especially those of the mouth and nose.
When paracoccidioidomycosis affects the lymphatic system, generalized swelling of lymph nodes (lymphadenopathy) may occur in many areas of the body, especially in the neck and the underarm area (axilla). Infected lymph nodes may become painful and produce pus (suppuration).
In visceral paracoccidioidomycosis, other organs of the body may also be infected including the liver, spleen, and/or intestines. The adrenal glands may be particularly susceptible to this infection. Chronic adrenal involvement may cause abnormally low levels of adrenal hormones.
Paracoccidioidomycosis is caused by infection with a fungus known as Paracoccidioides brasiliensis. Many cases of this disease occur years after airborne fungal spores are inhaled, although the period of latency is not always this long.
The fungus is thought to exist in soil as a mold, and infection occurs following inhalation of spores (conidia). In the lungs, the fungus is converted to yeasts that may spread to other sites. Some of those exposed are able to resist this process and the infection is stopped. However, in others the fungus goes on to cause disease in one or more parts of the body.
Paracoccidioidomycosis sometimes occurs in patients whose immune systems have been weakened (immunocompromised), including those with AIDS.
Paracoccidioidomycosis is a rare fungal disease. For reasons that are not clearly understood, its chronic adult form affects males 15 times more frequently than it does females. Most affected people are between the ages of 20 and 50 years. The subacute juvenile form of the disorder affects males and females equally.
Paracoccidioidomycosis is rare in the United States but can occur in people who have visited, or migrated from, South and Central America.
The diagnosis of paracoccidioidomycosis is usually made by examination of sputum or pus from infected individuals. If positive, microscopic examination will permit the identification of the responsible fungus, Paracoccidioides brasiliensis. Diagnosis may also be made by the examination of tissue samples (biopsy specimens) from the lungs, skin, and/or lymph nodes. The diagnosis is confirmed when samples of infected tissue are grown in the laboratory (cultured) and eventually test positive for the presence of Paracoccidioides brasiliensis.
Blood tests may also be useful for the diagnosis of paracoccidioidomycosis, but they cannot distinguish between active and past infection. Skin tests are available but may not be reliable. Chest x-rays of affected individuals may show patchy areas of fungal infection (infiltration).
Antifungal drugs are the most effective therapeutics for paracoccidio-idomycosis. Among these are itraconazole, ketoconazole and fluconazole. Amphotericin B may be given to patients with severe disease who cannot tolerate other medications. Sulfonamides suppress the symptoms and halt the progress of the disease, but do not eliminate the fungus from the body.
The antifungal drugs ketoconazole, itraconazole, and fluconazole are often prescribed as treatments for paracoccidioidomycosis and other systemic fungal infections. Studies indicate that these drugs may be as effective as amphotericin B. More study is needed to determine the long- term safety and effectiveness of ketoconazole and itraconazole for the treatment of paracoccidioidomycosis.
Scientists have discovered an antigen in the blood of people (Gp43) with Paracoccidioidomycosis that they believe is produced in response to infection with Paracoccidioides brasiliensis. It is hoped that this discovery will lead to improved blood tests for the diagnosis of this disease.
Research on tropical diseases is ongoing. The development of vaccines is also being investigated. For more information, contact the World Health Organization (WHO) listed in the Resources section below.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
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Email: [email protected]
For information about clinical trials sponsored by private sources, contact:
Fauci AS, Braunwald E, Isselbacher KJ, et al., eds. Harrison’s Principles of Internal Medicine. 14th ed.McGraw-Hill Companies. New York, NY; 1998:1160.
Mandell GL, Bennett JE, Dolan R, eds. Mandell, Douglas and Bennett’s Principles and Practice of Infectious Diseases. 4th ed. Churchill Livingstone Inc. New York, NY; 1995:2386-89.
dos Santos JW, Debiasi RB, Miletho JN, et al. Asymptomatic presentation of chronic pulmonary paracoccidioidomycosis: case repiort and review. Mycopathologia. 2004;157:53-57.
Miyaji M, Kamei K. Imported mycoses: an update. J Infect Chemother. 2003;9:107-13.
Trent JT, Kirsner RS. Identifying and treating mycotic skin infections. Adv Skin Wound Care. 2003;16:122-29.
San-Blas G, Nino-Vega G, Iturriaga T. Paracoccidioides brasiliensis and paracoccidioidomycosis: molecular approaches to morphogenesis, diagnosis, epidemiology, taxonomy and genetics. Med Mycol. 2002;40:225-42.
Ellis D. Amphotericin B: spectrum and resistance. J Antimicrob Chemother. 2002;49 Suppl 1:7-10.
Bethlem EP, Capone D, Maranhao B, et al. Paracoccidioidomycosis. Curr Opin Pulm Med. 1999;5:319-25.
FROM THE INTERNET
Boxwalla AA. Paracoccidioidomycosis. emedicine. Last Updated: June 25, 2002. 11pp.
Paracoccidioidomycosis. Merck Manual. ©2004 Merck & Co., Inc. 2pp.
Paracoccidioidomycosis. Merck Manual Second Home Edition Online. ©2004 Merck & Co., Inc. 2pp
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