Paracoccidioidomycosis

Paracoccidioidomycosis
Classification and external resources
Paracoccidioides brasiliensis
ICD-10	B41
ICD-9	116.1
DiseasesDB	29815
eMedicine	med/1731
MeSH	D010229

Paracoccidioidomycosis (also known as "Brazilian blastomycosis,"^[1] "South American blastomycosis,"^[1],"Lutz-Splendore-de Almeida disease"^[2] and "Paracoccidioidal granuloma"^[3]:320) is a mycosis caused by the fungus Paracoccidioides brasiliensis. Sometimes called South American blastomycosis, paracoccidioidomycosis is caused by a different fungus than that which causes blastomycosis.

Agent

P. brasiliensis is a thermally-dimorphic fungus distributed in Brazil and South America. The habitat of the infectious agent is not known but appears to be aquatic. In biopsies the fungus appears as a polygemulating yeast with a pilot's wheel-like appearance.

Disease

Paracoccidioidomycosis is a systemic mycosis caused by the dimorphic fungus Paracoccidioides. It frequently involves mucous membranes, lymph nodes, bone and lungs. Unlike other systemic mycoses, it can cause disease in immunocompetent hosts, although immunosuppression increases the aggressiveness of the fungus. Also uniquely, it rarely causes disease in fertile-age women, probably due to a protective effect of estradiol.^[4]

Primary infection is thought to be autolimited and almost asymptomatic as histoplasmosis or Valley Fever. In young people, there is a progressive form of the disease (akin of tuberculous septicemia in tuberculous priminfection) with high prostrating fever, generalized lymphadenopathy and pulmonary involvement with milliary lesions. This juvenile form has a more severe prognosis even with treatment. The most common form is the so called adult form of paracoccidioidomycosis that is almost certainly a reactivation of the disease.

Painful lesions with a violaceous hue in lips and oral mucosa are common as is cervical lymphadenitis teeming with polygemulating yeasts in the biopsy. In this form, differential diagnosis must be made with mucocutaneous leishmaniasis, yaws and TB.

Pulmonary involvement is also common, it starts as lobar pneumonia or pleurisy but without remission at ninth day; the patient remains febrile, coughs, loses weight and the X rays reveal milliary shadows throughout lung fields. Other organs can be involved, like bones, meninges, arteries and spleen but this is very rare.

Diagnosis is made with a biopsy of affected tissue, this shows the characteristic helm-shaped yeasts and culture shows the agent. Serology is also used in endemic areas.

Treatment

Sulfonamides are the traditional remedies to paracoccidiodomycosis. They were introduced by Oliveira Ribeiro and used for more than fifty years with good results. The most used sulfa drugs in this infection are sulfadimethoxime, sulfadiazine and co-trimoxazole. This treatment is generally safe but several adverse effects can appear, the most severe of which are the Stevens Johnson Syndrome and agranulocytosis. Similarly to tuberculosis treatment, it must be continued for up to 3 years to eradicate the fungus, and relapse and treatment failures aren't unusual.

Antifungal drugs like amphotericin B or ketoconazole are also effective in clearing the infection but they are very expensive compared with sulfonamides.

During therapy fibrosis can appear and a surgery be needed to correct this. Other possible complication is Addisonian crisis. The death rate is approximately ten percent.

Discovery

Lutz-Splendore-de Almeida disease^[2] is named for the physicians Adolfo Lutz,^[5] Alfonso Splendore,^[6] and Floriano Paulo de Almeida,^[7][8] who first characterized the disease in Brazil in the early 20th century.

See also

• North American blastomycosis
• List of cutaneous conditions

References

1. ^ ^{a b} Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007), Dermatology: 2-Volume Set, St. Louis: Mosby, ISBN 1-4160-2999-0 
2. ^ ^{a b} Lutz-Splendore-de Almeida disease at Who Named It?
3. ^ James, William D.; Berger, Timothy G.; et al. (2006), Andrews' Diseases of the Skin: clinical Dermatology, Saunders Elsevier, ISBN 0-7216-2921-0 
4. ^ Severo LC, Roesch EW, Oliveira EA, Rocha MM, Londero AT (June 1998), "Paracoccidioidomycosis in women", Rev Iberoam Micol 15 (2): 88–9, PMID 17655417, http://www.reviberoammicol.com/pubmed_linkout.php?15p88. 
5. ^ Lutz A (1908), "Uma mycose pseudococcidioidica localizada no boca e observada no Brasil. Contribuicao ao conhecimento das hypoblastomycoses americanas." (in Portuguese), Imprensa médica (Rio de Janeiro) 16: 151–163 
6. ^ Splendore A (1912), "Zimonematosi con localizzazione nella cavita della bocca osservata nel Brasile" (in French), Bulletin de la Société de pathologie exotique (Paris) 5: 313–319 
7. ^ De Almeida FP (1928), "Lesoes cutaneas da blastomicose en cabaios experimentalmente infeetados" (in Portuguese), Anais da Faculdade de medicina de Univesidade da São Paulo 3: 59–64 
8. ^ de Almeida FP, da Silva Lacaz C (1942), "Micoses broco-pulmonares" (in Portuguese), Comp. Melhoramentos (São Paulo): 98 pages 

External links

• Overview at University of Adelaide