Case 6: ”Kutis Porcelana ”. “Kutis Porcelana” Strawberry is an 8-year old very...

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Case 6:Case 6:”Kutis ”Kutis

PorcelanaPorcelana””

“Kutis Porcelana”

Strawberry is an 8-year old very fair-complexioned girl planned by her beauty-contest conscious mother to become a future Mutya ng Pilipinas, brought to the hospital because of pruritic red spots on her chest, back and arms. Three days earlier, she complained of low-grade fever (37.80C), headache, anorexia and generalized body malaise. On PE, she had numerous vesicular lesions on an erythematous base scattered on the back, chest and arms. The rest of the physical findings were essentially normal.

Guide Questions

1. What conditions present with vesiculopustular eruptions?

2. How is the diagnosis of Varicella achieved?3. What is the treatment for Varicella?4. What are the complications of Varicella?5. Which high risk patients should receive VZIG to

prevent Varicella?6. What is responsible for prevention and control of

Varicella?

Q1: What conditions

present with vesiculopustular

eruptions?

Conditions that present with Vesiculopapular/pustular eruptions

Varicella– Herpes zoster

Herpes simplex infections Hand, foot and mouth Syndrome (Coxsackie A16) Impetigo Insect bites Molloscum contagiosum Scabies

1. Varicella

A benign but highly contagious infection in immunocompetent individuals

Contagious from a few days before the skin lesions appear to 5-7 days after the appearance of the lesions

Varicella-zoster infection

A prodromal period is usually absent.The rash & constitutional symptoms occur simultaneously

The rash is characterized by:1. Rapid evolution of macules to

papules to vesicles to crusts2. Central distribution of lesions

which occur in crops3. Presence of the lesions in all

stages in any one anatomic area4. Involvement of the scalp and

mucous membranes5. Eventual crusting of nearly all of

the lesionsKrugman’s Infectious Disease of Children

Varicella-zoster infection

• The pleomorphic rash of varicella evolves very rapidly through stages of macule, papule, vesicle, pustule and crust.

• Skin lesions are first seen on the body and inner aspects of the thighs but spread quickly to the face, scalp and proximal parts of the limbs

Varicella rashes

Lesions emerge in crops at irregular intervals up to a week

The rash is heaviest on the trunk and diminishes in intensity towards the periphery

Pruritus may be troublesome during the first few days

Varicella

Crusting to scarring

2. Herpes Zoster (Shingles)

Due to reactivation of latent VZV Herpes Zoster, Herpes Simplex

and Vaccinia occur without any prodromal period

The lesions of herpes zoster are unilateral and distributed along the line of affected nerves.The vesicles are grouped together and tend to become confluent

Herpes Zoster

Predominantly a disease of the middle-aged & elderly. Less than 5% of attacks occur in children below 10 yrs.

When zoster develops in very young children, there is frequently a history of an attack in the mother during pregnancy.

Post-herpetic neuralgia is seldom a problem in children

3. Herpes simplex Infection

Congenital herpes simplex group

of vesicles on arms and back

Herpes simplex labialisEczema herpeticum

4. Hand, Foot and Mouth Disease

5. Impetigo contagiosa & Impetigo bullosa

Impetigo contagiosa:– The classic lesion begin as

erythematous papules in traumatized areas. They quickly evolve into honey-colored crusted plaques with surrounding erythema

– GAS and S. aureus are the chief causative agent

Impetigo bullosa :– Exclusively staphylococcal in origin– The characteristic lesion are caused

by epidermolytic toxin

Impetigo contagiosa

Impetigo bullosa

Impetigo contagiosa & Impetigo bullosa

They differ from chickenpox in appearance and distribution

They do not appear in crops, do not involve the mucous membranes of the mouth and are not accompanied by constitutional symptoms

The lesions of impetigo commonly involve the nasolabial area because of the tendency for a child to scratch with contaminated fingers

6. Molluscum Contagiosum

A viral skin infection caused by a large DNA virus (poxvirus).

The lesions are grouped pinpoint papules that increase in size.

They are off white or pinkish-tan and rounded and may have a central umbilication

Most frequently occurs in children There are no associated systemic

symptoms No specific therapy is required

7. Insect bites, Papular urticaria

Local reactions may resemble infectious exanthems or may be secondarily infected.

The lesions do not have a typical vesicular appearance and do not involve the scalp or mucus membranes

8. Scabies

Typical lesions are papules, vesicles, and burrows that tend to occur in some areas of predilection e.g. interdigital folds

Because of itching and scratching, the rash becomes excoriated, crusted, eczematized and secondarily infected

The differential points of scabies are the same as those of insect bites. The burrows between the fingers and toes and the microscopic identification of Sarcoptes scabiei help confirm the diagnosis

Q2:How is the diagnosis

ofVaricella achieved?

VZV Diagnosis

Diagnosis is usually made clinically– The presence of a herpesvirus can be demonstrated

by a Tzanck smear that demonstrates inclusions– Infection can be confirmed by acute and

convalescent titers of VZV antibody

Q3:What are the

complicationsof Varicella?

Complications

Secondary bacterial superinfection particularly with Group A streptococcus, the most frequent

Encephalitis Viral or bacterial Pneumonia

– Recognized chiefly in otherwise healthy adults and immunocompromised children

Hepatitis DIC (rare) with purpura fulminans**Complications occur more frequently in

immunocompromised patients and adults

Neonatal Chickenpox

Severe disseminated and fatal varicella in 5-10 day old infants resembling that in leukemic children may occur in offspring whose mothers have varicella 5 days before delivery to 2 days after delivery.

Maternal varicella near term: effect on the newborn infant (50 cases)

Onset Effect

Maternal varicella, 5 or

more days before delivery;

baby’s varicella age 0-4

days

Maternal varicella. 4 days or

less before delivery;

Baby’s varicella, age 5-10

days

27 of 27 survived

16 of 23 survived (7 died of diseminated varicella. 2 had severe disease with survival

Gershon AA.,”Infections of the fetus and newborn infant”,1975

Varicella in the immunocompromised

Neonates whose mothers are not immune and patients with leukemia may suffer severe, prolonged or fatal chickenpox (Disseminated or Progressive Varicella)

The mortality rate of untreated primary infection in immunocompromised children is 7-14% and may approach 50% in adults

Q4:What is the treatment

for Varicella?

Treatment

IV Acyclovir should be given to immunocompromised patients with Varicella or Herpes zoster

Oral acyclovir given to healthy children with varicella within 24 hrs of the rash results in diminution and duration of skin lesions. Should be considered in adolescents and adults with varicella

VZIG should be given with 96 hrs of exposure to susceptible high risk patients for severe or complicated Varicella

Q5:Which High RiskPatients shouldreceive VZIG to

prevent Varicella?

High Risk Patients who should receive VZIG

Immunocompromised, susceptible children without history of varicella or varicella immunizations (who have had household exposure, shared a hospital room or played indoors for at least 1 hour with children with contagious VZV)

Normal susceptible adults, especially pregnant women Newborn infant of a mother who had onset of Chickenpox within 5

days before or48 hours after delivery Hospitalized premature infant (>28 wks gestation) whose mother

has not had chickenpox All hospitalized premature infants <28 wks gestation or weighing

<1,000 gms.regardless of maternal hx of varicella

Q6: What is responsible for prevention and

control of Varicella?

Prevention and control of Varicella

Varicella vaccine for susceptible children aged 12 months to 12 years

Live-attenuated preparation of serially propagated and attenuated wild Oka strain

Dose: 0.5 ml subcutaneously(recommended) or IM, one dose for children <12 yrs or younger and 2 doses 4-8 weeks apart for individuals older than 12 yrs of age

– 95% immunogenic for immunized healthy children between 12 mos and 12 yrs of age with humoral and CMI response

– 78-82% after 1 dose and 99% after 2 doses for people 13 yrs or older

– Duration of immunity: at least 11 yrs (USA); 20 yrs (Japan studies)

Key Learning Points

Although a benign disease, Varicella can have disastrous effects on the immunocompromised

The herpesviridae family e.g. Herpes zoster manifest a propensity for latency or inactivity but may be reactivated following immunosuppression or other stress factors

Maternal varicella can result in a congenital varicella syndrome, Herpes zoster or neonatal varicella which can have disastrous or fatal effects on the fetus

The most effective prevention against Varicella is thru immunization with a live attenuated vaccine

VZIG is an effective tool to prevent dire consequences to the unprotected host

Case 6:Case 6:”Kutis ”Kutis

PorcelanaPorcelana””