Progressive vaccinia
| Progressive vaccinia | |
|---|---|
| Other names | 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, Clostridioides 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”) |
| Usual onset | 11 days to 6.5 weeks |
| Duration | Long-lasting |
| Causes | Injection by the vaccinia virus (genus: orthopoxvirus) as a countermeasure for smallpox |
| 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" |
| 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.