Blastomycosis south american B40.3

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Almeida disease; Brazilian blastomycosis; Costa Rica bastomycosis; Cutaneous primary paracoccidioidomycosis; Granuloma paracoccidioides; Lutz-Splendore Almeida disease; Paracoccidioides brasiliensis; Paracoccidioidomycosis; Paracoccidiomycosis; South American blastomycosis

History
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Lutz, 1908; Splendore, 1912; de Almeida, 1928

Definition
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In South America occurring, chronically progressive, deep systemic mycosis especially with infestation of the mouth and nose mucosa as well as the adjacent facial skin in form of purulent granulomatous foci.

Pathogen
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Paracoccidioides brasiliensis (= Blastomyces brasiliensis), a dimorphic fungus that occurs in soil and dust.

Occurrence/Epidemiology
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Spread over the entire South American continent, especially in Brazil and Latin America. Common in rural areas, therefore especially among farm and agricultural workers. Common in patients with immunosuppression (e.g. HIV infection).

Etiopathogenesis
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Infection with P. brasiliensis. The fungus lives in the soil and is transmitted to humans by inhalation through contaminated dust. Infection of healthy people through contact with infected people is not possible.

Manifestation
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Mostly 20 to 30 years of age, mostly white people. Men are 10 times more frequently affected than women.

Clinical features
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Initially infestation of the lungs, oral and nasal mucosa and the regional lymph nodes.

Beginning with cough and thoracic pain, followed by development of painful, ulcerative, granulomatous lesions of the oral and nasal mucous membranes. Facial skin affection mainly centrofacial: granulomatous, nodular, verruciform papules; later large, chronic, possibly mutating ulcers. Swelling and possibly purulent melting inflammation of the cervical lymph nodes is characteristic.

Hematogenous spread of the pathogens may involve bone, spleen, adrenal glands, CNS.

Diagnosis
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Microscopic and cultural pathogen detection from sputum, pus, bioptic material (by multilocular sprouting of the fungus in the yeast phase formation of characteristic "steering wheel" forms).

Therapy
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Amphotericin B (e.g. Amphotericin B) i.v. 1 mg/kg bw/day for 4-6 weeks, in severe cases liposomal Amphotericin B (e.g. AmBisome) initially 1 mg/kg bw i.v.; if necessary, increase gradually to 3 mg/kg bw i.v. until complete healing.

Alternatively: Itraconazole 100-400 mg/day in 1-2 ED p.o. or Ketoconazole 200-600 mg/day p.o. over 6 months, even if lesions heal earlier.

Progression/forecast
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Untreated lethal course.

Literature
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  1. Benard G et al (2000) Paracoccidioidomycosis: a model for evaluation of the effects of human immunodeficiency virus infection on the natural history of endemic tropical diseases. Clin Infect Dis 31: 1032-1039
  2. Cacere CR et al (2002) The role of apoptosis in the antigen-specific T cell hyporesponsiveness of paracoccidioidomycosis patients. Clin Immunol 105: 215-222
  3. de Almeida SM et al (2002) Anti-gp43 antibodies in the cerebrospinal fluid of patients with central nervous system involvement by paracoccidioidomycosis. At J Clin Pathol 118: 864-868
  4. de Almeida FP (1928) Lesoes cutaneas da blastomicose en cabaios experimentalmente infeetados. To Fac Med S Paulo 3: 59-64
  5. Garcia Bustinduy M et al (2000) Cutaneous primary paracoccidioidomycosis. J Eur Acad Dermatol Venereol 14: 113-117
  6. Gomes GM et al (2000) PCR for diagnosis of paracoccidioidomycosis. J Clin Microbiol 38: 3478-3480
  7. Lutz A (1908) Uma mycose pseudococcidioidica localizada no boca e observada no Brasil. Contribuicao ao conhecimento das hypoblastomycoses americanas. Impr Med Rio 16: 151-163
  8. Marques da Silva SH et al (2003) Detection of circulating gp43 antigen in serum, cerebrospinal fluid, and bronchoalveolar lavage fluid of patients with paracoccidioidomycosis. J Clin Microbiol 41: 3675-3680
  9. Maymo Arganaraz M et al (2003) Paracoccidioidomycosis and larynx carcinoma. Mycoses 46: 229-232
  10. Negroni R et al (1993) Paracoccidioidomycosis (South American blastomycosis, Lutz's mycosis). Int J Dermatol 32: 847-859
  11. Ollague JM et al (2000) Paracoccidioidomycosis (South American blastomycosis) successfully treated with terbinafine: first case report. Br J Dermatol 143: 188-191
  12. Splendore A (1912) Zimonematosi con localizzazione nella cavita della bocca osservata nel Brasile. Bulletin of the Société de pathologie exotique (Paris) 5: 313-319

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020