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Paracoccidioidomycosis

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Synonyms of Paracoccidioidomycosis

Disorder Subdivisions

General Discussion

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.

Symptoms

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.

Causes

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.

Affected Populations

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.

Related Disorders

Symptoms of the following disorders can be similar to those of Paracoccidioidomycosis. Comparisons may be useful for a differential diagnosis:

Blastomycosis or North American Blastomycosis is a systemic fungal infection caused by Blastomyces dermatitidis. It occurs primarily in the southeastern and Mississippi valley areas of the United States. Symptoms initially include fever, chills, cough, heavy sweating, and/or difficulty breathing (dyspnea). Other symptoms may develop later and involve the lungs, skin, bones, joints, kidneys, and/or central nervous system. Chronic infection of the lungs may lead to pneumonia. If left untreated, Blastomycosis can have life-threatening complications. (For more information on this disorder, choose "Blastomycosis" as your search term in the Rare Disease Database.)

Tuberculosis is a bacterial disease caused by Mycobacterium tuberculosis or Mycobacterium bovis. It is characterized by an initial period without symptoms. Pneumonia may be the first symptom in some people with Tuberculosis. Other symptoms may include coughing, weight loss, night sweats, fever, and/or fatigue. Chest x-rays typically reveal cavities in the lungs caused by chronic infection. The major symptom at this stage is coughing and the production of phlegm. (For more information on this disorder, choose "Tuberculosis" as your search term in the Rare Disease Database.)

Nocardiosis is a rare infectious disease caused by the bacteria Nocardia asteroides and is characterized by acute inflammation of the lungs (pneumonia) and abscesses in the lungs. Symptoms include the profound loss of appetite (anorexia), generalized weakness, cough, and/or chest pain. Some affected individuals may also have abscesses in the brain, kidneys, intestines, and/or other organs of the body. Symptoms associated with brain abscesses may include severe headaches and other neurological difficulties. (For more information on this disorder, choose "Nocardiosis" as your search term in the Rare Disease Database.)

Toxoplasmosis is an infectious disorder that is caused by a parasite (Toxoplasma gondii) that is found in the feces of cats. This infection is found worldwide and may be either acquired or transmitted to a fetus from an infected mother. When the disorder is acquired, the symptoms are similar to mononucleosis or involve lesions of the lungs, liver, heart, skin, muscle, brain, and spinal cord membranes. Lesions are often accompanied by inflammation and in some cases hepatitis. Acute cases are often characterized by rash, high fever, chills, and fatigue. To avoid this disease, pregnant women and people with an impaired immune system should wear a protective mask when emptying litter boxes containing cat feces. The prognosis for the acquired forms of Toxoplasmosis is usually good with treatment, and complications are uncommon. Without treatment this disorder may persist for many months. (For more information on this disorder, choose "Toxoplasmosis" as your search term in the Rare Disease Database.)

Standard Therapies

Diagnosis
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).

Treatment
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.

Investigational Therapies

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
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

Paracoccidioidomycosis Resources

Organizations:

References

TEXTBOOKS
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.

REVIEW ARTICLES
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.
www.emedicine.com/med/topic1731.htm

Paracoccidioidomycosis. Merck Manual. ©2004 Merck & Co., Inc. 2pp.
www.merck.com/mrkshared/mmanual/section13/chapter158/158e.jsp

Paracoccidioidomycosis. Merck Manual Second Home Edition Online. ©2004 Merck & Co., Inc. 2pp
www.merck.com/,,he/sec17/ch197/ch197i.html

Report last updated: 2009/04/08 00:00:00 GMT+0