Kutis Porcelana

Case 6: ”Kutis Porcelana”

“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. 2. 3. 4. 5. 6.

What conditions present with vesiculopustular eruptions? How is the diagnosis of Varicella achieved? What is the treatment for Varicella? What are the complications of Varicella? Which high risk patients should receive VZIG to prevent Varicella? 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 

 3. 4. 5. 6. 7.

A prodromal period is usually absent.The rash & constitutional symptoms occur simultaneously The rash is characterized by: Rapid evolution of macules to papules to vesicles to crusts Central distribution of lesions which occur in crops Presence of the lesions in all stages in any one anatomic area Involvement of the scalp and mucous membranes Eventual crusting of nearly all of the lesions

Krugman’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

Eczema herpeticum

Herpes simplex labialis

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 honeycolored crusted plaques with surrounding erythema GAS and S. aureus are the chief causative agent

Impetigo contagiosa

Impetigo bullosa : – –

Exclusively staphylococcal in origin The characteristic lesion are caused by epidermolytic toxin

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 

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

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 of Varicella 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 complications of 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 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

Effect 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 Risk Patients should receive VZIG to prevent Varicella?

High Risk Patients who should receive VZIG 

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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  

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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: ”Kutis Porcelana”