DEADLY DUST

By Danielle Timothy RN, BSN Epidemiologist

The Story of Coccidioidomycosis

Northern Argentina, 1889: 33-year old soldier Domingo Escurra is examined by medical personnel for what was thought to be a spider bite. The lesion had worsened and other lesions sprouted even after he had applied tobacco and used a penknife to create incisions. Escurra was diagnosed with lupus vulgaris (painful tuberculosis skin lesions) at the military hospital, but the prescribed treatment was ineffective and he was transferred to another hospital where he was diagnosed with mycosis fungoides (skin lymphoma). Treatment was again unsuccessful. In 1891, as the lesions began to take over Escurra’s extremities and trunk, he was admitted to the University Hospital Clinics in Buenos Aires, where he came under the care of intern Alejandro Posadas. Accepting the mycosis fungoides diagnosis, Posadas published a detailed and groundbreaking description of his patient’s disease, and followed the case until Escurra’s death seven years later. Domingo Escurra’s head was preserved, along with a hand and foot, as medical specimens. Meanwhile, during the same time period, two farm laborers who had immigrated from the Azores were hospitalized in San Francisco with skin lesions. Microscopic examinations showed the presence of spherical organisms which doctors named “coccidioides”. Treatment for the disease, applied under chloroform anesthesia, was described as follows: “All the affected areas … were most thoroughly scraped with a sharp spoon. The raw surfaces were then vigorously scrubbed with a bristle brush and an abundance of bichlorite solution … was applied.” Both patients eventually died from the illness.

At the turn of the century, this disease was finally properly classified by Professor William Ophüls, who had studied the case of another Azorian farm immigrant who had also recently died in a San Francisco hospital. He noted mold growth in lung tissue and the likelihood of airborne spread, and named the disease caused by the pathogenic mold “coccidioidal granuloma.” In 1948, while visiting an anatomy museum of the Buenos Aires Medical School in Argentina, a doctor encountered a disfigured human head preserved in a jar of formaldehyde. It had multiple skin lesions, but no identifying information. He recognized it from the well-known description of Domingo Escurra’s fatal illness published by Alejandro Posadas over fifty years prior. Escurra’s hand and foot were recovered later from another museum where they had been labeled ‘tuberous leprosy’. The specimens were tested and found to have actually been infected with coccidioidal granuloma, making Escurra the first known patient infected with what we now call coccidioidomycosis. Coccidioidomycosis, or “cocci” for short, is also referred to as valley fever. It is a disease caused by coccidioides fungus. These fungal spores are carried in dust particles and are found in the soil of areas that have low rainfall, high summer temperatures, and moderate winters. The fungus is found in Southwest USA, Mexico, Central and South America, and the Southwestern United States, including Southwest Utah. Cocci does not spread between people or animals, but is inhaled as airborne particles after soil is disturbed by wind, construction work, farming, digging, off-road vehicle use, biking, and hiking. Up to fifty percent of people who are infected develop flu-like or pneumonia-like symptoms one to four weeks after exposure. Some symptoms associated with the disease include coughing, chest pain, fever, headache, muscle and joint aches, rash, fatigue, shortness of breath, and sputum production that can last weeks to months. The rash is usually what individuals remember. It presents itself as painful or tender with slightly elevated bumps - either blue or brown - located on their chest, back, or extremities. The rash can also present as red and raised and may look like blisters or pointed pimple-like bumps. Those with weakened immune systems are at greatest risk of complications, while some studies have shown that people (especially men) of African American or Asian descent are at highest risk for contracting the disease. Cocci usually infects the lungs, but can spread throughout the body years after the primary infection. Those who have this disseminated disease (about 1%) can form abscesses in the skin, bone, central nervous system, joints, and tissues. The mortality rate is .07% in those infected with cocci but increases in patients who are elderly or immunocompromised and is 12-17% in cases of disseminated disease. Cocci meningitis is almost always fatal within two years if not treated. Early treatments for cocci, such as those mentioned in the cases of Domingo Escurra and the farm workers in California, also included topical applications of volatile oils and nystatin, x-ray therapy, and amputation of the affected extremities (“the only curative intervention”). It wasn’t until 1953 that soil obtained from the Orinoco River Valley in Venezuela was found to secrete anti-fungal properties which paved the way for effective treatment. Today, mild cases of cocci usually resolve on their own. Oral antifungal medications given over the course of 3-6 months can shorten the length of illness and prevent complications. More serious cases usually require treatment through intravenous medication. In some situations, surgical removal of cavities in the lungs or the draining of abscesses becomes necessary. Relapsing is possible, but rare, and most exposed individuals develop lifelong immunity. Currently there is not a vaccine for the disease. Coccidioidomycosis causes an estimated 15% to 30% of community-acquired pneumonias in areas like California and Arizona, and the disease has likely been widely under-recognized. Fortunately, healthcare providers in endemic regions are gradually becoming more aware of cocci and have testing available to confirm infections in their patients.

Mertensia alpina Bluebells bloom in mountainous meadows and aspen groves, blooming from late June to early October.