Blastomycosis, north 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)

Blastomycosis; Chicago disease; (e) Gilchrist disease; Gilchrist (Rixford) disease; Gilchrist's disease; North American blastomycosis; Zymonematosis

History
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Gilchrist, 1896

Definition
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Chronic, deep systemic mycosis with primary lung infection (by inhalation of the pathogens), occurring mainly in North America (Mississippi basin, east and south of the USA). Haematogenous scattering leads to secondary involvement of multiple organs including the skin. Occurs also as an opportunistic infection in various diseases. Basic diseases.

Pathogen
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Blastomyces dermatitidis (called Ajellomyces dermatitidis in the sexual stage), is a dimorphic fungus that can grow as a yeast and mould. Infections occur in humans and animals (Ditmyer H et al. 2011). In humans, infection usually occurs through inhalation of spores present in the soil. Very rarely, e.g. in the case of injuries in laboratories, infection is possible by penetration of the pathogen into the skin (so-called inoculation blastomycosis).

Occurrence/Epidemiology
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The fungus is endemic in parts of eastern North America, particularly northern Ontario, southeastern Manitoba, Quebec south of the St. Lawrence River, parts of the U.S. Appalachian Mountains and interconnected eastern mountain ranges, the western shore of Lake Michigan, the state of Wisconsin and the entire Mississippi Valley, including the valleys of some major tributaries such as the Ohio River. In addition, Blastomyces dermatitidis is rare in Africa both north and south of the Sahara desert, as well as on the Arabian Peninsula and the Indian subcontinent. Human-to-human transmission has only been reported in very few cases.

Manifestation
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Mostly adults, especially men (often agricultural workers).

Localization
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Face and extremities

Clinical features
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  • After an incubation period of 1-3 weeks symptoms of pulmonary infestation: cough, sputum, irregular fever, emaciation. Asymptomatic courses in about half of the cases.
  • Subsequently, further organ involvement through haematogenic scattering:
    • Skin lesions (80% of cases): Preferably on the extremities and face single or multiple, ulcerated, granulomatous papules with a verrucous-crustose surface as well as larger nodules with a tendency to ulceration, reaching into the subcutis. Progressing along the edges and central healing results in arched, flat, granulomatous, purulent plaques with raised edges.
    • Rare osteomyelitis of the trunk skeleton, skull and long tubular bones.
    • Infestation of the male reproductive organs.
    • Infestation of the mucous membranes of the mouth and nose.
    • Infestation of the CNS and the urogenital system.
    • In inoculation blastomycosis (direct inoculation of the pathogen when the skin is injured), formation of a solitary ulcer surrounded by a rough edge with accompanying lymphangitis.

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

Detection of antibodies in serum.

Possibly animal experiment.

Differential diagnosis
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Therapy
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  • Amphotericin B (e.g. Amphotericin B) i.v. 1 mg/kg bw/day over 4-6 weeks or liposomal amphotericin B (e.g. AmBisome®) initial 1 mg/kg bw i.v.; if necessary, increase gradually to 3 mg/kg bw i.v.
  • Alternatively: Fluconazole (e.g. Diflucan 50) 1 cps/day for 10 weeks (less toxic). Surgical removal of abscesses, removal of necrotic tissue.
  • Alternatively itraconazole for a longer period of time.

Progression/forecast
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Untreated chronic, often lethal course; spontaneous healing is possible.

Literature
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  1. Assaly RA et al (2003) Disseminated blastomycosis. J Am Acad Dermatol 48: 123-127
  2. Bradsher RW et al (2003) Infect Dis Clin North Am 17: 21-40
  3. Ditmyer H et al (2011) Mycotic mastitis in three dogs due to Blastomyces dermatitidis. J Am Anim Hosp Assoc 47:356 - 358.
  4. Gilchrist TC (1896) A case of blastomycetic dermatitis in man. Johns Hopkins Hosp Rep 1: 269-283
  5. Guinn DJ et al (2016) Fungal osteomyelitis with vertebral reossification. Int J Surg Case Rep 19:1-3.
  6. Court H (2019). Blastomyces dermatitidis. In: Hof H, Schlüter D, Dörries R, ed. dual series Medical Microbiology. 7th, completely revised and extended edition. Stuttgart: Thieme S 508
  7. Leavell UW Jr (1965) Cutaneous North American blastomycosis and black dots. Arch Dermatol 92: 155-156
  8. Martynowicz MA et al (2002) Pulmonary blastomycosis: an appraisal of diagnostic techniques. Chest 121: 768-773
  9. Pappas PG et al (2003) Blastomycosis: Gilchrist's disease revisited. Curr Clin Top Infect Dis 22: 61-77
  10. Sanghvi Y et al (2017) Blastomyces dermatitidis peritonitis complicating peritoneal dialysis. Semin Dial 30: 453-455.
  11. Wilkerson A et al (2003) Sweet's syndrome-like blastomycosis. Am J Dermatopathol 25: 152-154
  12. Zampogna JC et al (2003) Primary cutaneous north american blastomycosis in an immunosuppressed child. Pediatric dermatol 20: 128-130

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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