University of Alabama at Birmingham

2002 Case #2

Universidad Peruana Cayetano Heredia
 
The following case was seen on the Medicine Service of Cayetano Heredia Hospital in Lima by the 2002 Gorgas Course participants.
Image for 02/08/02History: 42 yo with onset of painful lip swelling 2 years ago, with gradual progression to facial and mucosal lesions shown in the digital images.  No fever, weight loss or other constitutional symptoms.  Treated for P. vivax malaria in 2000 with choloroquine and for a skin ulcer diagnosed clinically as leishmaniasis with pentavalent antimony in 1992.

Epidemiology: Farmer born in the high Andes but living for most of his life in Chanchamayo in the high jungle on the eastern side of the Andes.

Physical Examination: Afebrile.  Abnormalities restricted to face, buccal mucosa, tongue, and teeth as shown clockwise in digital image.  No adenopathy or organomegaly.

Labs/X-ray: Hematocrit 32.  WBC 8.3, N differential.  Arterial blood gases, urinalysis, liver function all normal.  Normal chest x-ray.



University of Alabama at Birmingham

2002 Case #2
Diagnosis and Discussion

Universidad Peruana Cayetano Heredia
(Links to Other 2002 Cases are at bottom of this page)
Diagnosis: Paracoccidioidomycosis due to chronic infection with Paracoccidioides brasiliensis.
Discussion: The major differential diagnosis includes mucocutaneous leishmaniasis due to Leishmania brasiliensis infection in light of the previous cutaneous ulcer on the hand compatible with leishmaniasis and the propensity of this organism to recur on mucosal surfaces months to years later.  In general however, oral lesions of paracoccidioidomycosis are painful while those of leishmaniasis are completely painless despite widespread destruction of tissue.  In addition, tongue lesions are unusual with leishmaniasis.  The infiltrative skin lesions on the face can also be confused with those of Balamuthia mandrillaris, a newly described species of free-living amebas described mostly in Perú but also in the US and other countries in the Americas.

The diagnosis was made by simple KOH preparation of a mucosal scraping which showed typical pilot-wheel shaped organisms consisting of spherical cells 10-40 microns in diameter with a thick birefrigent cell wall surrounded by several peripheral buds.  Direct scrapings will be positive in up to 90% of cases of paracoccidioidomycosis with oral lesions.  A lip biopsy also confirmed the diagnosis.

Paracoccidioidomycosis, also known as South American blastomycosis is found in humid forested or lush green areas of the Americas from Southern Mexico south to Uruguay and Argentina.  It appears to be most common in Brazil.  The exact habitat of the organism is unclear but transmission is described as being entirely by airborne inhalation.  However, we have observed cases with only oral lesions apparently associated with the use of tree leaves contaminated with fungal spores as toothpicks.  Similarly the use of leaves as toilet paper has resulted in anal infections that can be confused with anal cancer.  Primary pulmonary infection may be asymptomatic and self-limited but even with treatment will produce at least moderate pulmonary fibrosis.  Rural adult males agricultural workers between 30-60 years of age are most affected by the infection.  Travelers spending less than 6 months in an endemic area are unlikely to acquire paracoccidioidomycosis.

Extrapulmonary disease is found in over 70% of cases and may involve skin (facial areas in our patient), mucous membranes, lymph nodes, adrenals, abdominal organs and CNS (in 10%).  Oropharyngeal lesions are most common and typically present as painful ulcerative lesions.  There is often a red infiltrated hard base with exuberant heaped up hemorrhagic granulomatous lesions which may appear papillomatous (as seen on the buccal mucosa in our patient).  In contrast to leishmaniasis, the ulcers often bleed easily.  Edema of the lips and involvement of the gingival is common as seen in the images presented.  Bacterial superinfection of ulcerative lesions is more common that with oral ulcers due to mucocutaneous leishmaniasis.  15% of our patients have concomitant pulmonary TB.  This patient was sputum AFB negative X3.

Sulfonamides, ketoconazole, itraconazole, and amphotericin B are all effective therapies. Amphotericin should be reserved for severe cases.  While itraconazole 100 mg/day for 6 months or so is regarded as the treatment of choice, in the developing world setting ketoconazole is likely equally effective and is usually less than half the cost.  However, 12 months of therapy with ketoconazole is generally recommended.  Paracoccidioidomycosis is always a severe infection that may relapse even after prolonged treatment so always needs to be treated and followed up aggressively.