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CoccidioidomycosisB38.9

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

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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HistoryThis section has been translated automatically.

Wernicke and Posadas, 1892

DefinitionThis section has been translated automatically.

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

PathogenThis section has been translated automatically.

Coccidioides immitis, a dimorphic fungus.

Occurrence/EpidemiologyThis section has been translated automatically.

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.

EtiopathogenesisThis section has been translated automatically.

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.

LocalizationThis section has been translated automatically.

Face, neck, capillitium; also extremities and trunk.

Clinical featuresThis section has been translated automatically.

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.

HistologyThis section has been translated automatically.

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

DiagnosisThis section has been translated automatically.

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 diagnosisThis section has been translated automatically.

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

TherapyThis section has been translated automatically.

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.

Progression/forecastThis section has been translated automatically.

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.

AftercareThis section has been translated automatically.

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

LiteratureThis section has been translated automatically.

  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
  4. Kim A, Parker SS (2002) Coccidioidomycosis: case report and update on diagnosis and management. J Am Acad Dermatol 46: 743-747
  5. Mathew G et al (2003) Relapse of coccidioidomycosis despite immune reconstitution after fluconazole secondary prophylaxis in a patient with AIDS. Mycoses 46: 42-44
  6. Meier F et al (2004) Coccidioidomycosis. Two case reports with discussion of current diagnostic and therapeutic issues. dermatologist 55: 1143-1149
  7. Panackal AA et al (2002) Fungal infections among returning travelers. Clin Infect Dis 35: 1088-1095
  8. Polesky A et al (1999) Airway coccidioidomycosis--report of cases and review. Clin Infect Dis 28: 1273-1280
  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

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer