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Potentially life-threatening emergency
Progressive vaccinia - Smallpox Vaccination
See also in: Skin
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Potentially life-threatening emergency

Progressive vaccinia - Smallpox Vaccination

See also in: Skin
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Contributors: Art Papier MD, Vince Fulginiti MD
Other Resources UpToDate PubMed

Synopsis

Progressive vaccinia is the most severe complication of vaccinia (smallpox) vaccination. It is almost always life-threatening.

Progressive vaccinia occurs because of an immune defect in the vaccinated individual or in a susceptible contact of a vaccinee. Nearly all instances have been in those with a defined cell-mediated immune (CMI) defect (T-cell deficiency). In patients with CMI deficiency but intact antibody (B-cell) function, progressive vaccinia occurs, but is a less extensive disease, often limited to progression in the skin without viremic spread.

Virus gains entry into the blood at an early stage in patients with close to totally deficient immune systems and implants in distant skin sites and in multiple organs. Secondary skin lesions follow the same pattern as the vaccination site lesion, each expanding in situ. Local and systemic bacterial infection as well as parasitic (P. carinii and fungi) infections can ensue with progressive disease.

Untreated or unsuccessfully treated patients succumb in what appears to be toxic or septic shock. Death occurs in nearly all individuals with profound CMI defects.

In the past, individuals survived when their immune function improved coincident with the withdrawal of immunosuppressive therapy or spontaneous improvement in their underlying disease. Aggressive administration of vaccinia immune globulin (VIG) then resulted in cures. Patients with milder degrees of depression of CMI responded to aggressive VIG therapy.

Codes

ICD10CM:
T88.1XXA – Other complications following immunization, not elsewhere classified, initial encounter

SNOMEDCT:
56140006 – Progressive vaccina

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Differentiate from severe bacterial infection, where the patient lacks a history compatible with immune defect and has a vigorous inflammatory response. Differentiate from severe varicella (chickenpox) and disseminated herpes simplex virus (HSV) infections by noting a lack of a lesion at the primary vaccination site or, if vaccinated, the character of the lesions (superficial vesicles, in varying stages, with typical distribution). Smallpox (eradicated and only a bioterrorism threat) may be confused with progressive vaccinia in an individual who has been vaccinated and exposed.

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Therapy

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References

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Last Updated: 07/16/2018
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Potentially life-threatening emergency
Progressive vaccinia - Smallpox Vaccination
See also in: Skin
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Progressive vaccinia : Fever, Individual recently immunized with vaccinia, Patient appears systemically ill - toxic
Clinical image of Progressive vaccinia
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