Coccidioidomycosis B38.9

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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California disease; California Disease; Coccidioidal granuloma; Desert-fever; desert rheumatism; Desert rheumatism; Granuloma coccidioidales; Granuloma coccidioides; San Joaquin fever; San Joaquin Valley Fever; Valley fever

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Wernicke and Posadas, 1892

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Endemic, acute or chronic deep mycosis with primary infection of the lungs and hematogenic spread to different organs.

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Coccidioides immitis, a dimorphic fungus.

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Among blacks and Asians 10-15 times more often than among Caucasians. More common among immunocompromised people, especially those infected with HIV.

Endemic in semi-arid areas (e.g. Southern California, Arizona, New Mexico, Southwest Texas, Northern Mexico, Northern Argentina and Paraguay). Incidence there 10-20/100,000 inhabitants/year.

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Inhalation of fungal elements (arthroconidia) with formation of a primary complex in the lung. In 40% of the exposed patients, a primary coccidioidomycosis then develops in the lungs, which either heals spontaneously or becomes the starting point for haematogenic scattering.

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Face, neck, capillitium; also extremities and trunk.

Clinical features
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Incubation period: 10-18 days.

Primary pulmonary form: flu-like symptoms with fever and feeling of illness, pleuritic chest pain, possibly bloody sputum. Anorexia, weight loss (mainly 3-9 kg).

Allergic skin symptoms in the primary infection: Often erythema nodosum or erythema exsudativum multiforme.

In about 0.5% of cases haematogenous scatter with infestation of other organs, especially the skin and subcutis, bones (osteomyelitis), CNS and meninges.

Integument: Often first on the face, nasolabial folds, neck or capillitium. Also deep-seated panniculitic nodules, abscess and fistula formation.

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Tuberculoid granulation tissue with abscesses; detection of mature spherules with numerous endospores. Also immature (yeast-like) spherules.

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Pathogen detection in native preparation of pus and sputum, bronchoalveolar lavage, blood, urine or liquor. Histology.

Cultural pathogen detection: Grey-white, later darkening colonies covering the whole surface within 2-4 days.

Serology: specific antibody detection in immunocompetent individuals usually possible up to 2 years after initial infection by immunodiffusion or Western blot. In the acute stage, CBC is recommended as a supplementary method for quantification and titer control.

Intracutaneous skin test: The coccidioidin (e.g. Spherulin) skin test becomes positive within the first 4 weeks after onset of symptoms and usually remains positive for life - therefore not suitable for the detection of a florid infection!

Differential diagnosis
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Granulomatous processes of skin of other genesis; tuberculosis, blastomycosis; actinomycosis.

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In many cases clinically inapperent courses and healing without antimycotic therapy (in immunocompetent patients).
  • Cutaneous form: Azole is recommended especially for skin infections. Therapy of the 1st choice is Itraconazole (Sempera) 200-400 mg/day p.o. up to 6 months beyond clinical healing. Alternatively Fluconazole (e.g. Diflucan): Day 1: 400 mg p.o. as ED; from day 2: 200 mg p.o. as ED. Therapy duration: 3-6 months beyond clinical healing (control of transaminases!).
  • Disseminated form: Itraconazole (Sempera) 200-400 mg/day p.o. up to 6 months beyond clinical healing. In life-threatening cases Amphotericin B in high dosages: Initially 0.1 mg/kg bw/day i.v., then gradually increased to max. 1 mg/kg bw/day i.v. (max. up to 3 g/day).
    • Alternatively, liposomal amphotericin B (AmBisome) 1 mg/kg bw/day i.v., if necessary gradually increasing to 3 mg/kg bw/day.
    • Alternatively ketoconazole 400-1,600 mg/day i.v. or fluconazole 400-800 mg/day i.v.
    • Alternatively posaconazole: 2 times/day 400 mg (10 ml) p.o. (daily dose 800 mg) or 4 times/day 200 mg (5 ml) p.o. The duration of therapy depends on the severity of the underlying disease, recovery from immunosuppression if necessary and the clinical response.

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The disseminated form is a life-threatening disease that is lethal if left untreated. Dissemination can occur years after the primary affection of the lungs.

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Frequent relapses after discontinuation of therapy for systemic mycoses require years of clinical, mycoserological and possibly radiological controls.

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  1. Einstein HE et al (1993) Coccidiodomycosis: new aspects of epidemiology and therapy. Clin Infect Dis 16: 394-354
  2. Gabe LM et al (2017) Diagnosis and Management of Coccidioidomycosis. Clin Chest Med 38:417-433.
  3. Johnson RA (2000) HIV disease: mucocutaneous fungal infections in HIV disease. Clin Dermatol 18: 411-422
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  9. Posadas A (1892) Un nuovo caso de micosis fungoides con psorospermias. Anales dell Circulo médico Argentino (Buenos Aires) 15: 585-597
  10. Tsukadaira A et al (2002) Chronic coccidioidomycosis. Lancet 360: 977
  11. Wernicke RJ (1892) Pentastomas. Revista de la Sociedad Medica Argentina 1: 186-189 and Central Bulletin of Bacteriology and Parasitology 12: 859-861


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Last updated on: 29.10.2020