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ESSENTIALS OF DIAGNOSIS
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Appropriate epidemiologic setting, eg, exposure to animals or animal hides, or potential exposure resulting from an act of bioterrorism A painless cutaneous black eschar on exposed areas of the skin, with marked surrounding edema and vesicles Nonspecific flu-like symptoms that rapidly progress to extreme dyspnea and shock in association with mediastinal widening and pleural effusions on chest radiograph
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The death of a Florida photo editor from inhalational anthrax acquired from a letter deliberately contaminated with spores of Bacillus anthracis thrust this extremely rare infection into the public awareness Between September 18 and November 21 of 2001, there were 13 cases of cutaneous anthrax and 11 cases of inhalational anthrax associated with exposure to anthrax spores in contaminated mail Naturally occurring anthrax is a disease of sheep, cattle, horses, goats, and swine B anthracis is a gram-positive sporeforming aerobic rod Spores not vegetative bacteria are the infectious form of the organism These are transmitted to humans from contact with contaminated animals, animal products, or animal hides, or from soil by inoculation of broken skin or mucous membranes; by inhalation of aerosolized spores; or, rarely, by ingestion resulting in cutaneous, inhalational, or gastrointestinal forms of anthrax, respectively
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B Laboratory Findings
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Laboratory findings are nonspecific The white blood cell count initially may be normal or modestly elevated, with polymorphonuclear predominance and an increase in early
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forms Pleural fluid from patients with inhalational anthrax is typically hemorrhagic with few white cells Cerebrospinal fluid from meningitis cases is also hemorrhagic Gram stain of pleural fluid, cerebrospinal fluid, unspun blood, blood culture, or fluid from a cutaneous lesion may show the characteristic boxcar-shaped encapsulated rods in chains The diagnosis is established by isolation of the organism from culture of the skin lesion (or fluid expressed from it), blood, or pleural fluid or cerebrospinal fluid in cases of meningitis In the absence of prior antimicrobial therapy, cultures are invariably positive Cultures obtained after initiation of antimicrobial therapy may be negative If anthrax is suspected on clinical or epidemiologic grounds, immunohistochemical tests (eg, to detect capsular antigen), polymerase chain reaction assays, and serologic tests (useful for documenting past cutaneous infection) are available through the CDC and should be used to establish the diagnosis Any suspected case of anthrax should be immediately reported to the CDC so that a complete investigation can be conducted
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C Imaging Studies
The chest radiograph is the most sensitive test for inhalational disease, being abnormal (though the findings can be subtle) initially in every case of bioterrorism-associated disease Mediastinal widening due to hemorrhagic lymphadenitis, a hallmark feature of the disease, has been present in 70% of the bioterrorism-related cases Pleural effusions were present initially or occurred over the course of illness in all cases, and approximately three-fourths had pulmonary infiltrates or signs of consolidation
Differential Diagnosis
Cutaneous anthrax, despite its characteristic appearance, can be confused with a variety of other also uncommon or rare conditions such as ecthyma gangrenosum, rat-bite fever, ulceroglandular tularemia, plague, glanders, rickettsialpox, orf (parapoxvirus infection), or cutaneous mycobacterial infection Inhalational anthrax must be differentiated from mediastinitis due to other bacterial causes, fibrous mediastinitis due to histoplasmosis, coccidioidomycosis, atypical or viral pneumonia, silicosis, sarcoidosis, and other causes of mediastinal widening (eg, superior vena cava syndrome or aortic aneurysm or dissection) Gastrointestinal anthrax shares clinical features with a variety of common intra-abdominal disorders, including bowel obstruction, perforated viscus, peritonitis, gastroenteritis, and peptic ulcer disease
of concern for engineered drug resistance in strains of B anthracis used in bioterrorism or weaponized, ciprofloxacin is considered the drug of choice (Table 33 2) for treatment and for prophylaxis following exposure to anthrax spores Other fluoroquinolones activity against gram-positive bacteria (eg, levofloxacin, moxifloxacin) are likely to be just as effective as ciprofloxacin Doxycycline is an alternative first-line agent Combination therapy with at least one additional agent is recommended for inhalational or disseminated disease and in cutaneous infection involving the face, head, and neck or associated with extensive local edema or systemic signs of infection, eg, fever, tachycardia and elevated white blood cell count Anecdotally, four of the six survivors of the 2001 inhalational cases were treated with combinations that included both a fluoroquinolone and rifampin Single-drug therapy is recommended for prophylaxis following exposure to spores The required duration of therapy is poorly defined In naturally occurring disease, treatment for 7 10 days for cutaneous disease and for at least 2 weeks following clinical response for disseminated, inhalational, or gastrointestinal infection have been standard recommendations Because of concern about relapse from latent spores acquired by inhalation of aerosol in bioterrorism-associated cases, the initial recommendation was treatment for 60 days In 2001, the CDC offered one of two options for postal workers receiving prophylaxis for exposure to contaminated mail: (1) antibiotics for 100 days (fearing that even with 60 days of treatment late relapses might occur) or (2) vaccination with an investigative agent (three doses administered over a 1-month period) in conjunction with 40 days of antibiotic administration to cover the time required for a protective antibody response to develop Insufficient information exists to favor one recommendation over the other There is also an FDA-approved vaccine for persons at high risk for exposure to anthrax spores The vaccine is cell-free antigen prepared from an attenuated strain of B anthracis Multiple injections over 18 months and an annual booster dose are required to achieve and maintain protection Existing supplies have been reserved for vaccination of military personnel The prognosis in cutaneous infection is excellent Death is unlikely if the infection has remained localized,
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