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Smallpox case definitions

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      As described in “Guide A” of the “Smallpox Response Plan and Guidelines” (www.bt.cdc.gov/agent/smallpox/response-plan).

      Surveillance for a disease that does not currently exist anywhere in the world presents unique challenges. The goal of pre-outbreak (pre-event) smallpox surveillance is to recognize the first case of smallpox, should it ever occur, without generating excessive numbers of false alarms, unnecessarily disrupting the health care and public health systems, or increasing public anxiety. In the absence of known smallpox disease, the predictive value of a positive smallpox diagnostic test is extremely low; therefore, testing to rule out smallpox should be limited to cases that fit the clinical case definition in order to lower the risk of obtaining a false-positive test result. It is neither feasible nor desirable, in the pre-event scenario, to perform laboratory testing for suspected cases that do not meet the clinical case definition.

      Thus, in the absence of smallpox disease in the world, the suggested approach to surveillance relies on a highly specific clinical case definition, which is focused on identifying the classic case presentation (ordinary type) of smallpox. Before eradication, classic (ordinary type) smallpox generally accounted for approximately 90% of smallpox cases in previously unvaccinated individuals and 70% of cases that occurred in previously vaccinated individuals who were no longer fully protected by vaccination.

      Because the likelihood of reintroduction of smallpox is extremely low, and acknowledging that there are many other causes of vesicular and pustular rash illnesses, healthcare providers evaluating such cases should also familiarize themselves with diseases that can be confused with smallpox (e.g., varicella, herpes simplex, drug reactions, erythema multiforme), as well as the clinical manifestations of smallpox disease. In this way, in the unlikely event of a smallpox case, the disease will be clearly and quickly recognized.

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