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Paracoccidioidomycosis

Practice Essentials

Paracoccidioidomycosis is a fungal infection endemic to South and Central America, most notably Brazil, Argentina, Colombia, and Venezuela (see the image below). It is caused by the thermally dimorphic fungi Paracoccidioides brasiliensis and Paracoccidioides lutzii.
Although the infection is usually subclinical, the fungus can also cause chronic and severe disease.

Approximate distribution of paracoccidioidomycosis

Approximate distribution of paracoccidioidomycosis in North, Central, and South America, based on case reports.

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The 2 general clinical categories of paracoccidioidomycosis are (1) an acute/subacute form (juvenile paracoccidioidomycosis) and (2) a chronic form (adult paracoccidioidomycosis).

Signs and symptoms

Juvenile form

Lymphadenopathy and hepatosplenomegaly

Systemic symptoms – Include fever, weight loss, and malaise; present in most patients.

Symptoms related to lymph node enlargement, suppuration, and sinus tract formation

Multiple skin lesions

Mucous membrane and respiratory symptoms – These are unusual

Adult form

Primary lung infection – Cough (productive or nonproductive), dyspnea, malaise, fever, and weight loss are common symptoms

Chronic pulmonary sequelae – Develop in one third of patients; can include pulmonary fibrosis, bullae, and emphysematous changes that can contribute to pulmonary hypertension and cor pulmonale in 5% of cases.

Mucous membrane involvement – Occurs in 50% of patients with acute pulmonary infection; includes laryngeal and pharyngeal lesions

Oral lesions – May be associated with nasal and pharyngeal ulcers (Aguiar-Pupo stomatitis) and with mandibular or cervical lymph node enlargement

Cutaneous lesions – Caused by hematologic dissemination from the lungs; occur in 25% of patients; crusted papules, ulcers, nodules, plaques, and verrucous lesions are typical

Lymphadenopathy – Most common in the cervical region

Diagnosis

The diagnosis of paracoccidioidomycosis is most commonly made by visualization of the yeast cells in tissue, wet preparations (eg, sputum), or superficial scrapings (eg, skin lesions). Serological tests are available in areas of highest endemicity. In patients with active paracoccidioidomycosis, chest radiography reveals interstitial infiltrates (in 64% of cases) or mixed lesions with linear and nodular infiltrates.

Management

Systemic antifungal medications are the mainstay of medical management in paracoccidioidomycosis. Supportive measures and hospitalization may be warranted for patients with severe disease. If present, anemia and nutritional deficiencies should be treated. Antifungals effective against paracoccidioidomycosis include the following:

Triazoles – Itraconazole, voriconazole, and posaconazole; itraconazole is considered the drug of choice for paracoccidioidomycosis

Imidazoles – Ketoconazole

Sulfonamides – Sulfadiazine, trimethoprim/sulfamethoxazole

Amphotericin B – Reserved for severe cases of paracoccidioidomycosis that are refractory to other forms of therapy

Surgery

Occasionally, specific surgical treatments are warranted for life- or organ-threatening disease, such as neurosurgical procedures to relieve granuloma-induced spinal cord compression or hydrocephalus. Reconstructive surgery is sometimes needed to alleviate fibrotic sequelae.

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