Paracoccidioidomycosis

Paracoccidioidomycosis
Paracoccidioides brasiliensis
Specialty Infectious disease Edit this on Wikidata

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

Symptoms and signs

As in the majority of paracoccidioidomycosis cases, pulmonary involvement results in shortness of breath, a productive cough and hemoptysis, as well as general symptoms of weight loss, fever and fatigue.[4] Visually, lesions (as pictured) are often present, most commonly on the face.

Cause

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.[5]

Mechanism

Lesions due to Paracoccidioidomycosis on the face of a Brazilian child

Paracoccidioidomycosis is a systemic mycosis caused by the dimorphic fungus Paracoccidioides brasiliensis. Strong evidence indicates this fungus infects the host through the respiratory tract.[6] 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.[7]

Primary infection is thought to be autolimited and almost asymptomatic as histoplasmosis or coccidioidomycosis (valley fever). In young people, a progressive form of the disease (akin of tuberculous septicemia in tuberculous priminfection) occurs 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, the so-called adult form of paracoccidioidomycosis, 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 the ninth day; the patient remains febrile, coughs, and loses weight, and the X-rays reveal milliary shadows throughout lung fields. Other organs can be involved, such as bones, meninges, arteries, and spleen, but this is very rare.

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

Diagnosis

Diagnosis is often made by visualization of yeast cells in tissue, or superficial scrapings. Radiography of the chest reveals interstitial infiltrates in the majority of cases.[8][9]

Treatment

Sulfonamides are the traditional remedies to paracoccidiodomycosis. They were introduced by Oliveira Ribeiro and used for more than 50 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 three years to eradicate the fungus, and relapse and treatment failures are not unusual.

Antifungal drugs such as amphotericin B or itraconazole and ketoconazole are more effective in clearing the infection, but are limited by their cost when compared with sulfonamides.During therapy, fibrosis can appear and surgery may be needed to correct this. Another possible complication is Addisonian crisis. The mortality rate in children is around 7-10%.[5]

Epidemiology

Paracoccidioidomycosis has been reported as an autochthonous disease from southern Mexico to northern Argentina. No cases have been reported from Belize and Nicaragua in Central America, or from Chile, French Guiana, Guiana, and Suriname in South America. Paracoccidioidomycosis is prevalent in Brazil, Colombia, Venezuela, and Argentina, and is classically associated with individuals from rural areas. The typical patient is a man aged 30 to 50 years.[6]

History

Lutz-Splendore-de Almeida disease[2] is named for the physicians Adolfo Lutz,[10] Alfonso Splendor (1871–1953), an Italo-Brazilian Parasitologist[11] and Floriano Paulo de Almeida (1898–1977), a Brazilian Pathologist specializing in Pathologic Mycology (Study of Infectious Fungi),[12][13] who first characterized the disease in Brazil in the early 20th century.

See also

References

  1. 1 2 Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007), Dermatology: 2-Volume Set, St. Louis: Mosby, ISBN 1-4160-2999-0
  2. 1 2 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. ↑ F. Franco, Marcello; Del Negro, Gildo; Lacaz, Carlos da Silva; Restrepo-Moreno, Angela (1994). Paracoccidioidomycosis. Boca Raton: CRC Press. ISBN 9780849348686. OCLC 28421361.
  5. 1 2 Pereira, Ricardo M.; Tresoldi, Antonia Terezinha; da Silva, Marcus T. N.; Bucaretchi, Fábio (January 2004). "Fatal disseminated paracoccidioidomycosis in a two-year-old child". Revista Do Instituto De Medicina Tropical De Sao Paulo. 46 (1): 37–39. ISSN 0036-4665. PMID 15057333.
  6. 1 2 Marques, SA (Nov–Dec 2012). "Paracoccidioidomycosis". Clinics in dermatology. 30 (6): 610–5. doi:10.1016/j.clindermatol.2012.01.006. PMID 23068148.
  7. ↑ Severo LC, Roesch EW, Oliveira EA, Rocha MM, Londero AT (June 1998), "Paracoccidioidomycosis in women", Rev Iberoam Micol, 15 (2): 88–9, PMID 17655417.
  8. ↑ R Hospenthal, Duane (1 June 2017). "Paracoccidioidomycosis: Practice Essentials, Background, Pathophysiology". Medscape. Retrieved 8 September 2017.
  9. ↑ Marques-da-Silva, Silvia Helena; Messias Rodrigues, Anderson; de Hoog, G. Sybren; Silveira-Gomes, Fabíola; Pires de Camargo, Zoilo (2012-10-03). "Occurrence of Paracoccidioides lutzii in the Amazon Region: Description of Two Cases". The American Journal of Tropical Medicine and Hygiene. pp. 710–714. doi:10.4269/ajtmh.2012.12-0340. PMC 3516324. PMID 22927496. Missing or empty |url= (help); |access-date= requires |url= (help)
  10. ↑ Lutz A (1908), "Uma mycose pseudococcidioidica localizada no boca e observada no Brasil. Contribuicao ao conhecimento das hypoblastomycoses americanas.", Imprensa médica (in Portuguese), Rio de Janeiro, 16: 151–163
  11. ↑ Splendore A (1912), "Zimonematosi con localizzazione nella cavita della bocca osservata nel Brasile", Bulletin de la Société de pathologie exotique (in French), Paris, 5: 313–319
  12. ↑ De Almeida FP (1928), "Lesoes cutaneas da blastomicose en cabaios experimentalmente infeetados", Anais da Faculdade de medicina de Universidade de São Paulo (in Portuguese), 3: 59–64
  13. ↑ de Almeida FP, da Silva Lacaz C (1942), "Micoses broco-pulmonares", Comp. Melhoramentos (in Portuguese), São Paulo: 98 pages
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