Paracoccidioidomycosis

Paracoccidioidomycosis presents as chronic infection (90% of cases) with progressive pulmonary disease often with ulcerative and nodular lesions in the nose and mouth. Coffee growers (and other workers in contact with the soil) are affected in endemic areas throughout Central and South America.

CASES/YEAR
0 (US); 6,500 (Global)
CATEGORY
AGENT TYPE
Fungi (Endemic-Dimorphic)
OTHER NAMES
Paracoccidioides brasilensis infection; South American blastomycosis;
ACUITY
Subacute/Chronic
INCUBATION
1 month to many years; [CCDM]
INITIAL SYMPTOMS
Chronic infection (90% of cases) presents as progressive pulmonary disease often with ulcerative and nodular lesions in the nose and mouth. [Harrison ID, p. 1026] Progresses slowly over many years mimicking TB; [Cecil]
PRECAUTIONS
"Not transmitted from person to person." [CCDM, p. 442]
COMMENTS
FINDINGS:
Findings in the juvenile form include lymphadenopathy, hepatomegaly, and splenomegaly; Findings in the adult form include ulcers of the skin, mouth, and nose; difficulty swallowing; lymphadenopathy; respiratory problems (dyspnea, cough chest pain, sputum production, and hemoptysis); and brain lesions. [CCDM, p. 441-2] Pulmonary lesions occur in the middle and lower lung fields. Progressive fibrosis is the major cause of death. [Cohen, p. 297] Most infections are subclinical. Most symptomatic patients have chronic pulmonary disease with productive cough, weight loss, and dyspnea. [Guerrant, p. 582-3] Paracoccidioidomycosis can cause abdominal pain related to enlargement of the abdominal lymph nodes, liver, and spleen. [Merck Manual, p. 1576] Chest x-ray findings include infiltrates, nodules, cavitation, and hilar adenopathy. Patients may suffer from chest pain, productive cough, weight loss, fatigue, and fever. Infection of the adrenal glands was present in 95% of the cases in an autopsy series, and in a series of living patients, adrenal insufficiency was found in 48%. [ID, p. 2255-6] In about 65% of patients with active disease, the chest x-ray shows interstitial infiltrates. Some patients have hemoptysis. Ulcerations of the upper respiratory and GI tracts may cause dysphonia, dysphagia, diarrhea, and weight loss. Other lesions may occur in the spleen, liver, CNS, male genitourinary tract, bone, and bone marrow. The four main forms are subclinical (latent), residual, acute-subacute (children, young adults, and AIDS patients), and chronic progressive (90% of disease) [PPID, p. 3215-18] Jaundice is rare. [Guerrant, p. 582]

JUVENILE FORM:
In the acute or juvenile form seen in the young and patients with AIDS, there is widespread infection of the liver, spleen, lymph nodes, bone marrow, skin, and lungs, culminating in ARDS. [Cohen, p. 297] The juvenile form presents as fever, weight loss, eosinophilia, anemia, hepatosplenomegaly, and skin nodules/abscesses. [Guerrant, p. 582]

EPIDEMIOLOGY:
Coffee growers in Brazil, Columbia, and Venezuela are affected. Other workers in contact with the soil in endemic areas (farmers, laborers, and construction workers) are also at increased risk. There are endemic areas throughout Central and South America. The ratio of male to female patients is 15:1. Patients (usually older men from rural area) often develop symptoms many years after exposure. The main two forms of the disease are chronic adult (85% of cases) and juvenile (15% of cases). In juvenile patients, the disease is more acute and severe, and the target organ is the reticuloendothelial system (lymph nodes, liver, and spleen). In the chronic adult form, the target organ is the lungs with dissemination to other organs. [CCDM; Guerrant; Merck Manual; PPID]
DIAGNOSTIC
Wet mount or histology (budding yeasts); Culture; Serology; Relatively large yeast cell with translucent walls & surrounded by multiple buds resembling "pilot wheel"; [PPID] Takes up to 4 wks to grow; Use KOH or calcofluor fluorescent stain; [Cecil, 2045]
SCOPE
Tropical & subtropical Latin America from Mexico to Argentina, but absent in Chile and Caribbean Islands; Highest endemicity in Brazil and lowest in Colombia, Venezuela, Belize, Nicaragua, the Guianas, Ecuador, and Argentina; [CCDM]
SIGNS & SYMPTOMS
  • >fatigue, weakness
  • >fever
  • E dysphagia
  • E nasal ulcers
  • E stomatitis
  • G abdominal pain
  • G diarrhea
  • G hepatomegaly
  • G jaundice
  • H anemia
  • H eosinophilia
  • H lymphadenopathy
  • H splenomegaly
  • O oculoglandular syndrome
  • R chest pain
  • R cough
  • R dyspnea
  • R hemoptysis
  • R sputum production
  • S lymphadenitis, acute
  • S papules or plaques
  • S skin or subcutaneous nodule
  • S ulcer of skin
  • S warty growth of the skin
  • X cystic or cavitary lesions
  • X hilar lymphadenopathy
  • X lung infiltrates
  • *ARDS
  • *arthritis
  • *brain abscess or lesion
  • *encephalitis
  • *epididymo-orchitis
  • *hepatitis
  • *meningitis
  • *osteomyelitis
  • *pneumonia
  • *uveitis
  • *weight loss
ANTIMICROBIC

Yes

VACCINE

No

ENTRY
Inhalation
SOURCE
Soil or Dust (Ingesting or Inhaling)
RESERVOIR
RISK FACTORS
  • AIDS patients
  • Plow or excavate soil in endemic area
  • Travel to endemic area
TREATMENT
"Presently, itraconazole is considered the best choice for mild -to-moderate infections as it is effective in 95% of cases with low relapse rates and less toxic." [CCDM]
REFERENCES FOR CASES/YEAR
1.
2. (Global) >80% of cases are from Brazil; [Cecil, p. 2045] Most patients are rural men, middle aged or older. Most cases appear to be reactivations of latent infections acquired many years earlier. [Harrison iD, p. 1026] There are an estimated 3,000 to 10,000 new cases per year. [ID, p. 2254] Calculate: average of 3,000 and 10,000 = 6,500;