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Smallpox (Variola)

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Home - National Notifiable Diseases Surveillance System
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2004 Case Definition

Clinical Case Definition

An illness with acute onset of fever 101º F ( 38.3 º C) followed by a rash characterized by firm, deep seated vesicles or pustules in the same stage of development without other apparent cause. Clinically consistent cases are those presentations of smallpox that do not meet this classical clinical case definition: a) hemorrhagic type, b) flat type, and c) variola sine eruptione. (Detailed clinical description is available on the CDC web site, see URL: http://www.bt.cdc.gov/agent/smallpox/index.asp).


Laboratory Criteria

Polymerase chain reaction (PCR) identification of variola DNA in a clinical specimen,
OR
Isolation of smallpox (variola) virus from a clinical specimen (Level D laboratory only; confirmed by variola PCR).

Note: Indications for laboratory testing of patients with suspected smallpox should be followed as described in detail in Guide A of the CDC Smallpox Response Plan. Laboratory diagnostic testing for variola virus should be conducted in Level C or D laboratories only.

Case Classification*

Confirmed: case of smallpox that is laboratory confirmed, or a case that meets the clinical case definition that is epidemiologically linked to a laboratory confirmed case.

Probable: A case that meets the clinical case definition, or a clinically consistent case that does not meet the clinical case definition and has an epidemiological link to a confirmed case of smallpox.

Suspected: A case with a generalized, acute vesicular or pustular rash illness with fever
preceding development of rash by 1-4 days.

*Exclusion Criteria: A case may be excluded as a suspect or probable smallpox case if an alternative diagnosis fully explains the illness or appropriate clinical specimens are negative for laboratory criteria for smallpox.

Note: The smallpox case definition is to be used only during post-event surveillance. The case definition described in Guide A of the Smallpox Response Plan and Guidelines (Version 3) on the CDC bioterrorism preparedness website (URL: http://www.bt.cdc.gov/agent/smallpox/response-plan/index.asp) includes different criteria for a suspected case than the smallpox case definition the Council of State and Territorial Epidemiologists approved for use in the National Notifiable Diseases Surveillance System (NNDSS). The smallpox case definition on the CDC bioterrorism web site is more sensitive and less specific than the case definition for the NNDSS, in that a "suspect" case is defined as: "a case with febrile rash illness with fever preceding the development of rash by 1-4 days."

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