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Fungal Infections

Disease name and synonyms

Paracoccidioidomycosis (South American Blastomycosis)

Fungi responsible

Paracoccidioides brasiliensis and Paracoccidioides lutzii.

Disease description

Chronic pulmonary or disseminated disease is common (90% of cases) and may mimic malignancy or tuberculosis.  The most common general signs and symptoms are weight loss, lymph node enlargement, mucous lesions, weakness, and fever. Cough, usually productive, and dyspnoea are commonly reported respiratory symptoms. Pulmonary tuberculosis co-exists in 10-20% of those with pulmonary involvement. Other sites affected include the adrenal glands, the CNS, the cervical and submandibular lymph nodes, the intestines, bones or joints, the epididymis, the liver and the spleen. The multifocal or disseminated may present with pain during mastication, excess saliva and odynophagia. A few cases have been reported in AIDS.

Frequency and global burden

Endemic to all Latin America, especially Brazil. The larger number of cases has been reported in Brazil, Venezuela, Argentina, Uruguay, Ecuador, Colombia, Peru and Paraguay. Relatively few cases are reported from Bolivia or French Guiana. In Brazil probably ~ 3,500 annually, so <10,000 worldwide. The incidence may be declining because of changing agricultural practices and greater urbanization.

Underlying problems and at risk patients

Males are affected much more frequently than females, although a similar sex frequency is seen in pre-pubertal girls and post-menopausal women. Oestrogen blocks the mould to yeast transition in the fungus, preventing infection. AIDS increases the risk of more severe infection. Smoking probably increases the risk of chronic pulmonary disease.

Diagnostic testing

Chest radiographs shown bilateral disease in >90% of cases, usually occupying over a 1/3 of the lung area. Nodular and combined nodular/fibrotic appearances are typical of the chronic form of paracoccidioidomycosis.  Diffuse lung infiltrates are more typical of the acute form.

The diagnosis is usually made by culture from sputum or biopsy although the histologic appearances of P. brasiliensis are highly characteristic. Routine fungal culture of all specimens submitted for TB culture, increases the yield in endemic areas.

Treatment

Itraconazole is highly effective in non-immunocompromised patients. Amphotericin B is preferred in patients with severe pulmonary infection or in immunocompromised patients with disseminated disease. Sulfamethoxazole-trimethoprim is an alternative for those with chronic forms of disease with an 80% efficacy.

Outlook and prognosis

Overall estimated 5-30% mortality, sometimes as a result of co-morbidity such as tuberculosis or AIDS. Late diagnosis contributes to death. The main sequelae of paracoccidioidomycosis include worsening breathlessness due to pulmonary fibrosis and cavitation, adrenal gland dysfunction (~30%), dysphonia and/or laryngeal obstruction,  reduced mouth opening and epilepsy and/or hydrocephalus (~15% ).

 View Article pulmonary paracoccidioidomycosis 

Review article

Images

Pulmonary paracoccidioidomycosis showing bilateral cavitary lesions in the lid zones.

Involvement of the gums (mulberry-like stomatitis), a common feature of paracoccidioidomycosis, and one manifestation of disseminated disease (Courtesy of Angela Restrepo, Medellin).

Marked neck gland involvement in paracoccidioidomycosis, and a common feature of disseminated disease (Courtesy of Angela Restrepo, Medellin).

Disseminated Paracoccidioidomicosis. (Courtesy of Dra. M Melhem, Brazil)





Disseminated Paracoccidioidomicosis. (Courtesy of Dra. M Melhem, Brazil)

 

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