Makindo Medical Notes.com

Anyone who lives in or visits areas where Paracoccidioides lives can get paracoccidioidomycosis. Scientists estimate that less than 5% of patients with paracoccidioidomycosis die from the disease.

Worker from Brazil
Fever, lymph nodes, looking ill
Dry cough, dyspnoea
Tooth loss, gingiva won’t heal
Path shows budding pilot’s wheel     
@DrCindyCooper

About

• Dimorphic fungus Paracoccidioides
• Commonest route is inhalational
• Also known as Almeida disease, Lutz-Splendore-Almeida disease, Lutz’s mycosis, paracoccidioidal granuloma, South American blastomycosis

Geography

• Commoner in Men in outdoor rural occupations
• Mexico, Central and South America (most Brazil).
• Grows in the soil form in humid regions with a medium to a high rainy season, mild temperatures, and the presence of forests and rivers
• Habit of using twigs to clean teeth gives oral lesions

Aetiology

• Neutrophils and alveolar macrophages will tend to form granulomas depending on the immunological state of the patient and the T helper (TH) lymphocytes' response

Clinical

• Fatigue, weight loss, cough
• Prominent lymph nodes and enlarged Liver and spleen
• Bone marrow involvement with anaemia
• Lesions in mouth and throat - Gingival ‘mulberry’ lesions
• Pulmonary disease with cough and dyspnoea and fibrosis
• CNS involvement may result in meningoencephalitis

Investigations

• FBC, U&E, Ca, LFTs, CRP. CXR
• Biopsy of affected area will show fungal elements reveals spherical thick-walled yeast cells of variable size, with peripheral buds protruding from a central cell (pilot's wheel). It is often described as multipolar budding yeast with daughter cells resembling a “Mickey mouse head" or a “steering wheel."
• Culture: Sabouraud dextrose agar. takes 20-30 days to grow so not helpful
• Histologic findings: Methenamine silver stain or periodic acid Schiff stain are used to identify fungal elements in tissue samples. Lymph nodes may exhibit caseous necrosis. In the juvenile form, a diffuse tissue reaction is characteristic.
• Blood tests: Quantitative immunodiffusion testing is the most widely available assay in endemic regions and represents a reliable method with 84.3% sensitivity and 98.9% specificity.
• Lung Biopsy: Shows Yeast forms with Giant cells and Granuloma formation

Complications to prevent

• Pulmonary fibrosis. Malnourishment, anaemia
• Addison syndrome
• Bacterial or other fungal pathogen infections

Management: get expert help

• Para coccidioidomycosis does not spread from person to person
• First line: Itraconazole 100-400 mg OD
• Also: Voriconazole 400 mg BD on the first day then 200 mg BD
• Also: Ketoconazole 200-400 mg/day in adults for a course of 6 to 18 months
• Also: Amphotericin B or Trimethoprim/sulfamethoxazole (TMP/SMX) but needs 24 months or Sulfadiazine up to 4 g/day in adults
• Patients usually need treatment for about one year.

References

• Paracoccidioidomycosis