Vaccinia gangrenosum, Vaccinia necrosum or disseminated vaccinia
The patient required a skin graft on her upper left arm in order to correct the necrotic vaccination site, due to the onset of progressive vaccinia, formerly known as vaccinia gangrenosum.
Specialty
Dermatology
Symptoms
Malaise, fever, vomiting and tender, enlarged axillary lymph nodes; progresses to septic Pseudomonas aeruginosa, likely from a perirectal abscess, Clostridium difficile (bacteria), Staphylococcus aureus and cell-mediated immunodeficiency.
Complications
Necrosis of the injected part, exacerbating to gangrene and eventual amputation. Usually, the pocks tend to go away without scarring; however, the external and internal spread of the virus may have serious consequences in persons with eczema and other forms of atopic dermatitis, in these persons, defects of innate immunity and a high level of Th2 cell activity render the skin unusually permissive to the initiation and rapid spread of vaccinia infection (known as “eczema vaccinatum”)[1][2]
Usual onset
11 days to 6.5 weeks
Duration
Long-lasting
Causes
Injection by the vaccinia virus (genus: orthopoxvirus) as a countermeasure for smallpox[3]
Risk factors
People with cellular immunodeficiencies
Diagnostic method
Fever and headache, then progressive ulceration and necrosis of the injection site for smallpox, albeit the lack of inflammation is noted as the "hallmark of PV"[3]
Differential diagnosis
May initially be mistaken for leukemia
Prevention
Unknown
Treatment
Vaccinia Immune Globulin Intravenous (Human) (VIGIV), Emergency Investigational New Drug (E-IND) both administered orally and topically, (in this case ST-246); CMX001, a lipid conjugate of cidofovir and granulocyte colony-stimulating factor for the exiguous normal white blood cells;supportive care; skin graft
Medication
Imiquimod, and thiosemicarbazone
Prognosis
Lifelong
Frequency
every 1 or 2 in a million during routine vaccination during 1963-1968 for smallpox
Deaths
fatality rate: 15%
Progressive vaccinia is a rare cutaneous condition caused by the vaccinia virus, characterized by painless but progressive necrosis and ulceration.[4]
^Copeman, P. W. M.; Wallace, H. J. (10 October 1964). "Eczema Vaccinatum". BMJ. 2 (5414): 906–908. doi:10.1136/bmj.2.5414.906. PMC 1816899. PMID 14185655.
^Engler, Renata J.M.; Kenner, Julie; Leung, Donald Y.M. (September 2002). Written at Walter Reed Army Medical Center, Allergy-Immunology Department, Washington, DC, USA. "Smallpox vaccination: Risk considerations for patients with atopic dermatitis". Journal of Allergy and Clinical Immunology. 110 (3). Maryland Heights, Missouri, United States: jacionline.org: 357–365. doi:10.1067/mai.2002.128052. PMID 12209080.
^ abCenters for Disease Control Prevention (CDC) (22 May 2009). "Progressive Vaccinia in a Military Smallpox Vaccinee – United States, 2009". Morbidity and Mortality Weekly Report. 58 (19): 532–6. PMID 19478722.
^James, William D.; Elston, Dirk; Treat, James R.; Rosenbach, Misha A.; Neuhaus, Isaac, eds. (2019). "Progressive Vaccinia (Vaccinia Necrosum, Vaccinia Gangrenosum)". Andrews' Diseases of the Skin E-Book: Clinical Dermatology. Elsevier Health Sciences. p. 388. ISBN 978-0-323-55188-5.
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