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A 15-year-old Hispanic girl with “bump”
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A 15-year-old Hispanic female presented to the clinic with a ‘bump’ on her nose that had been draining pus for three days.
The patient was triaged as a “spider bite.” Examination revealed a papular lesion draining a small amount of pus. She was started on a standard dose of cephalexin for seven days and advised to follow-up if not improving.
She returned in two days with a new complaint of vomiting the night before. The lesion on her nose had improved but she appeared to be developing a new one on her forehead. She was advised to complete the course of cephalexin but if she was not tolerating it, to switch to amoxicillin-clavulanic acid (Augmentin, GlaxoSmithKline) for five days. She was advised to return to the clinic if she was not improving.
The patient returned approximately one month later. She was now wearing a dressing on her nose and the mother requested a referral to an ear, nose and throat specialist.
The patient’s mother was upset because she had been to a local emergency room (ER) five days after her last visit to our clinic. The patient was told that she had a MRSA infection, and she underwent drainage of a nasal abscess. She was discharged home on trimethoprim-sulfamethoxazole. The patient’s mother was angry because the ER had given her a different diagnosis from the diagnosis given to her in the clinic and the patient was still not improving.
The patient now had multiple lesions breaking out all over her body as well as fevers every other day and swelling of her arms and legs. The mother had also tried contacting the ER for further information but could not get anyone to give her follow-up results on the tests performed. Figures 1 and 2 (above) are of the patient’s appearance on third visit.
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Answer
The diagnosis is disseminated Coccidioides immitis.
C. immitis is a dimorphic fungus. It is the causative agent of coccidioidomycosis, or San Joaquin Valley fever, an endemic primary systemic mycosis that is asymptomatic in 95% of cases but also causes life-threatening infections in immunocompetent and immunocompromised hosts.
The infectious forms of this unique fungus are the multinucleate spores arthroconidia, which are thick, barrel-shaped structures that are released from the mold. Coccidioides species are most highly concentrated in the San Joaquin Valley of California and in south-central Arizona. The major burden of coccidioidomycosis falls on Arizona and California, with greater than 95% of all cases reported being from those two states. Kern County, which is located in the San Joaquin Valley, has been recognized as a site for hyperendemic coccidioidomycosis since the 1940s.
Primary infection occurs in the lungs. Primary infection, whether clinically apparent or asymptomatic, is often accompanied by lymphohematogenous dissemination of the fungus. Infection is sub-clinical in about 60% of people who have this illness, and most others have self-limited, primary pulmonary infections. Pulmonary complications occur in less than 5% and disseminated infections in less than 1% of people with this infection. The diagnosis is often belated, because the infection is not considered initially.
Dissemination of the infection to extrapulmonary sites usually occurs within several months after the primary pulmonary infection and rarely after one year. Persistent fever generally accompanies extrapulmonary disease. Individuals at highest risk are neonates and young infants, immunosuppressed patients, Filipinos, blacks, Native Americans and Hispanics.
Most skin infections represent extrapulmonary manifestations arising by spread from a primary pulmonary focus. The skin may be involved by immunologically-induced reactive eruptions or, more rarely, by dissemination of the organisms from the lungs. Lesions begin as papules or pustules that enlarge and can ulcerate. Verrucous plaques, subcutaneous cold abscesses, and wartlike lesions also occur. Lesions occur anywhere and are especially common on the face along the nasolabial folds. Although most skin infections result from dissemination, cutaneous lesions, accompanied by regional adenitis, rarely occur after accidental inoculation.
Symptoms of coccidiodomycosis are not distinctive. The main difficulty in diagnosis is failure to consider coccidioidomycosis. A careful travel history is needed for prompt recognition of the diagnosis, particularly in patients who reside in non-endemic areas. The mainstays of diagnosis are culture and serologic testing.
Less than 5% of people who have C. immitis require antifungal treatment. The decision to treat is based on the specific features and severity of the initial illness and the presence or absence of other risk factors that predict progressive dissemination, a poor outcome, or both. In general, treatment is required for patients who have primary or acquired immunodeficiency, fulminant infections, extrapulmonary manifestations, prolonged symptoms, or any combination of these conditions.
In patients with severe illness or rapid progression of disease, amphotericin B is used initially. In patients with disseminated disease, prolonged chemotherapy is always indicated. Recently, a number of new antifungal agents have been evaluated for their activity against C. immitis. Agents that have been shown to be potentially useful include voriconazole, caspofungin and posaconazole.
Disorders in cellular immunity or therapy with agents that impair cellular immunity are risk factors for aggressive and disseminated infection with C. immitis. Attempts to immunize individuals have been unsuccessful. In endemic regions, immunodeficient patients should be counseled about avoiding infection. All people, particularly those visiting or moving from nonendemic areas, should avoid exposure to activities that may aerosolize spores in contaminated soil. If such activities are unavoidable, respiratory filtration devices should be used.
For more information:
- Sabiha Hussain, MD, works for the pediatrics department at the Family Health Center/Community Action Partnership of Kern.
- Long S, Pickering L, Prober C. Principles and Practice of Pediatric Infectious Diseases. Second Edition.
- Saubolle M, McKellar P and Sussland D. Epidemiologic, clinical and diagnostic aspects of coccidioidomycosis. J Clin Microbiol. 2007; 45(1):26-30.
- Rosenstein N, Emery K, Werner S et al. Risk factors for Severe Pulmonary and Disseminated Coccidioidomycosis: Kern County, California, 1995-1996. CID. 2001; 32: 708-15
- Stevens D. Coccidioidomycosis. N Engl J Med. 332(16): 1077-1082.
- Dicaudo D, Yiannias J, Laman S et al. The exanthem of acute pulmonary coccidioidomycosis; clinical and histopathologic features of 3 cases and review of the literature. Arch Dermatol. 2006; 142: 744-746.
- Park D, Sohn J, Cheong H et al. Combination therapy of disseminated coccidioidomycosis with caspofungin and fluconazole. BMC Infect Dis. 2006;6:26
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