# Presentation: 186922

## Vaccinia Specific Adverse EventsKeratitis

**Notes:**
This picture demonstrates the residual corneal scarring and low grade immune keratitis after vaccinial complications.
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Annotation:  Residual vaccinial corneal scarring and low grade immune keratitis (inflammation of the cornea).
Source:  American Journal of Ophthamology.  Pepose & Langston, “Ocular Complications of...” (submitted for publication), photographs.  Deborah Pavan-Langston, MD, FACS, Harvard Medical School, Massachusetts Eye and Ear Infirmary.

## Vaccinia Specific Adverse EventsSevere Vaccinial Blepharitis

**Notes:**
Uncomplicated lesions are self-limited, resolving in about 3 weeks, and require no therapy.  If extensive body surface area is involved, or ocular vaccinia infection (without keratitis) or severe manifestation of inoculation has occurred, treatment with vaccinia immune globulin may speed recovery.
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Annotation:  Severe vaccinial blepharitis.
Source:  American Journal of Ophthamology.  Pepose & Langston, “Ocular Complications of...” (submitted for publication), photographs.  Deborah Pavan-Langston, MD, FACS, Harvard Medical School, Massachusetts Eye and Ear Infirmary.

## Vaccinia Specific Adverse EventsSevere Vaccinial Blepharitis

**Notes:**
This is the same patient as in the previous photo at day 10 after onset.  She was treated with one dose of VIG and vidarabine.
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Annotation:  The same patient as the previous slide.  Shows healing vaccinial blepharitis day 10.  Treated with vaccinia immune globulin x 1 and vidarabine.
Source:  American Journal of Ophthamology.  Pepose & Langston, “Ocular Complications of...” (submitted for publication), photographs.  Deborah Pavan-Langston, MD, FACS, Harvard Medical School, Massachusetts Eye and Ear Infirmary.

## Generalized Vaccinia

**Notes:**
Generalized vaccinia (GV) is characterized by a disseminated maculopapular or vesicular rash, frequently on an erythematous base, which usually occurs 6-9 days after first –time vaccination.  The rash spans the spectrum of vaccinial lesions, from maculopapules to vesicles.  Maculopapules can be mistaken for EM, when they are accompanied by a significant component of erythema. In other instances, the pearly vesicles of generalized vaccinia resemble the lesions of smallpox, however, GV does not follow the centrifugal distribution that is characteristic of smallpox. 
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Annotation:  Generalized vaccinia with a significant erythematous base in an infant.  Note the vaccination site at the left axilla and the well appearing child.
Source:  Fenner, et al.  “Smallpox and its Eradication.”  World Health Organization.

## Generalized Vaccinia

**Notes:**
The GV rash may be preceded by fever, but in general, the vaccinee appears minimally ill.  Lesions evolve following the time course of the vaccination site.  Lesions can be present anywhere on the body including the palms and soles and may be few or numerous. Generalized vaccinia may present as a regional form that is characterized by extensive satellite vesiculation around the vaccination site, or as an eruption localized to a body part (e.g. arm, leg), with no evidence of inadvertent inoculation.  There is also a mild form of GV, which presents with just a few scattered lesions.   
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Annotation:  The lesions of generalized vaccinia can, at times, be difficult to distinguish from variola (smallpox) infection.  Generalized vaccinia does NOT follow a centrifugal distribution which is characteristic of smallpox lesions.
Source:  J. Michael Lane, Teaching Slide set.  J.M. Lane, M.D.

## Generalized Vaccinia

**Generalized Vaccinia**

**Notes:**
Generalized vaccinia is self-limited in immunocompetent hosts.  These patients appear well and do not require VIG but may benefit from simple supportive care measures such as non-aspirin anti-inflammatory agents (NSAIDS) and oral antipruritics.  In the rare case where an immunocompetent individual appears toxic, VIG may have a role.  Generalized vaccinia is often more severe in individuals with an underlying immunodeficiency, and these patients may benefit from early intervention with VIG. 
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Annotation:  Generalized vaccinia in an apparently normal child.  Recovered without sequelae.
Source:  Photo courtesy of John M. Leedom, M.D.

## Generalized Vaccinia

**Notes:**
Here you see a regional generalized vaccinia.  This is characterized by an extensive halo of vesiculation around the vaccination site surrounded by a halo of vesicles. This differs from satellite lesions due to the number and extent of vesiculation.
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Annotation:  Regional Generalized vaccinia – characterized by an extensive halo of vesiculation around the vaccination site surrounded by a halo of vesicles.  This differs from satellite lesions due to the number and extent of vesiculation.
Source:  J. Michael Lane, Teaching Slide set.  J.M. Lane, M.D.

## Eczema Vaccinatum

**Notes:**
Eczema vaccinatum is a localized or generalized papular, vesicular, or pustular rash, which can occur anywhere on the body, with a predilection for areas of previous atopic dermatitis lesions. Onset of the characteristic lesions may be noted either concurrently with or shortly after the development of the local vaccinial lesions.   The eczema vaccinatum cases resulting from secondary transmission usually presented with skin eruptions approximately 5-19 days following the suspected exposure.  The EV lesions follow the same dermatological course as the vaccination site and confluent lesions may occur.   The rash is often accompanied by fever and lymphadenopathy and affected individuals are systemically ill.  Eczema vaccinatum tends to be more severe in a first-time vaccinees or unvaccinated contacts.
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References:
[i] Dixon CW. Smallpox. London: Churchill, 1962
[ii] Copeman PWM, Wallace HJ. Eczema vaccinatum. Br Med J 1964; 2:906.
Annotation:  A 22-year-old woman with eczema vaccinatum acquired from her boyfriend.  She became critically ill, with nearly total involvement of her body, and required thiosemicarbazones, as well as large doses of VIG.
Source:  J. Michael Lane, Teaching Slide set.  J.M. Lane, M.D.

## Eczema Vaccinatum

**Notes:**
Atopic dermatitis, irrespective of disease severity or activity, is a risk factor for developing eczema vaccinatum in either vaccinees or their close contacts, but no data exist to predict the absolute risk for this population.  The majority of primary care providers do not distinguish between eczema and atopic dermatitis when describing chronic exfoliative skin conditions, particularly in infants and young children.  Animal studies suggest an immunologic T-cell dysregulation predisposes individuals affected with atopic dermatitis to disseminated progressive papulo-vesiculo-pustular lesions even in intact skin.  
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References:
[i] Waddington E, Bray PT, Evans AD, Richards IDG. Cutaneous complications of mass vaccination in South Wales 1962. Trans St Johns Hosp Derm Soc 1964; 50:22-42.
[ii] Eriksson, G, Forsbeck, M.  Smallpox outbreak and vaccination problems in Stockholm, Sweden 1963. The assessment, and the vaccination, of patients with cutaneous disorders. Acta Med Scand Suppl Jan 1966; 464: 147-57
[iii] Engler RJ, Kenner J, Leung DY. Smallpox vaccination: Risk considerations for patients with atopic dermatitis. J Allergy Clin Immunol 2002; 110(3):357-365.
[iv] Beltrani VS. Clinical Features of Atopic Dermatitis. Immol Allergy Clin NA 2002; 22[1]. 2002.
Annotation:  The same woman from the side.
Source:  J. Michael Lane, Teaching Slide set.  J.M. Lane, M.D.

## Eczema Vaccinatum

**Notes:**
Eczema vaccinatum may be associated with systemic illness that includes fever and malaise.  Management includes hemodynamic support (as in sepsis) and meticulous skin care (as for burn victims).  Patients may require volume repletion and vigilant monitoring of electrolytes due to disruption of the dermal barrier.  Patients with eczema vaccinatum are at risk of secondary bacterial and fungal infections of the lesions and antibacterials and antifungals are indicated as necessary. 
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Annotation:  This is the same woman.  This picture shows residual scarring after the resolution of the systemic illness.
Source:  J. Michael Lane, Teaching Slide set.  J.M. Lane, M.D.

## Eczema Vaccinatum

**Notes:**
The next few slides show another example of eczema vaccinatum.  The 27-year-old previously vaccinated woman developed this moderately severe case 8 days after vaccination.  The individual lesions mixed with some confluence and umbilication are typical of vaccinia.
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Annotation:  Face of a 27-year-old previously vaccinated woman with moderately severe eczema vaccinatum 8 days after vaccination. The individual lesions with some confluence and umbilication are typical of vaccinia.
Source:  J. Michael Lane, Teaching Slide set.  J.M. Lane, M.D.