Paediatric%20Emergency%20Rounds%20Presenting%20Complaint:%20

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Paediatric Emergency RoundsPresenting
Complaint My child has a RASH

• Kelly Millar

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Overview of Topics

• Varicella in the immunocompromised host
• Fever and petechiae
• Acute exfoliating conditions

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What would you do?

• CASE 1
• 3 yo on prednisone (2 mg/kg/d) for nephrotic
  syndrome
• 5 yo sibling just diagnosed with chicken pox
• No prev Varivax, no hx of CP infection

• A) wait and see
• B) oral acyclovir
• C) IV acyclovir
• D) VZIG
• E) call ID!?!

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• CASE 2
• 3 yo on prednisone (2mg/kg/d) for nephrotic
  syndrome
• just diagnosed with chicken pox
• First lesion noted in past 24 hours

• A) wait and see
• B) oral acyclovir
• C) IV acyclovir
• D) VZIG
• E) call ID!?!

What would you do?
5

• CASE 3
• 3 yo on flovent (125ug) 2 puffs BID for asthma
  maintenance
• Just diagnosed with chicken pox
• First lesion noted in past 24 hours

• A) wait and see
• B) oral acyclovir
• C) IV acyclovir
• D) VZIG
• E) call ID!?!

What would you do?

• What if this child had received a 5 day course of
  prednisone (2mg/kg) 3 weeks ago?

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How well are physicians doing?

• General practice survey of the management of
  chickenpox appropriate targeting of antiviral
  therapy. Shepherd et al. Family Practice (2001)
  18(3)249-52
• Only 61.8 used antivirals when clear indication
  to do so
• 32.6 used antivirals when not indicated

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Varicella Exposure

• Defined as household, play, or classroom contact
  with person who has varicella or who develops
  varicella within 48 hours
• transmitted to 85-90 of susceptible household
  contacts
• May be acquired after close physical contact with
  person with zoster

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Varicella Prophylaxis Strategies

1. Varivax
2. VZIG
3. Acyclovir?

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Varicella Prophylaxis - Varivax

• Live attenuated
• Seroconversion rates
• Children gt 95 after 1 dose
• gt13yrs 80 after 1 dose, 99 after 2 doses
• 3-5 develop localized lesions, another 3-5
  generalized lesions (rarely transmissable)
• Evidence for post-exposure prophylaxis if given
  within 72 hrs post-exposure

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Varicella Prophylaxis - VZIG

• Highly effective when given within 96 hrs of
  exposure
• 10-15 have mild breakthrough infection
• Prolongs incubation period (if admitted, must be
  isolated for 35 days post exposure)

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Varicella Prophylaxis - Acyclovir

• 3 recent studies on po acyclovir prophylaxis for
  healthy household contacts
• Acyclovir given for 5-7 days starting on day 7
  post-exposure (coincides with viremia)
• Decreases the rate of clinical varicella by 65
• Problem Low varicella antibody titres
• ? Protection against reinfection
• ? Predisposition to early zoster

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Varicella Prophylaxis - Acyclovir

• No data for efficacy in immunocompromised
  patients
• ? Possible role if VZIG unavailable or if patient
  presents gt 96 hrs post-exposure

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Who needs post-exposure prophylaxis?

• No history of varicella PLUS one of
• Newborns whose mothers developed lesions from 5
  days prior to 48 hrs post delivery
• Pregnant women
• Immunocompromised high dose steroids in past 30
  days, chemotherapy, BMT, immunosuppressives,
  congenital cellular immunodeficiencies, HIV

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What about steroids?

• Low dose
• lt 1mg/kg/day prednisolone or equivalent
• lt 20 mg/day total prednisolone or equivalent
• Inhaled steroids
• High Dose
• gt 1 mg/kg/day or gt 20mg per day total of
  prednisolone or its equivalent
• Beware of anabolic steroid users

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What PEP should be used?

• In the immunocompromised?
• If present within 96 hours post-exposure
• GIVE VZIG
• If present after 96 hours post-exposure
• Consider acyclovir
• Possible role for combined VZIG po acyclovir
• In the healthy patient?
• May offer Varivax within 72 hours of exposure

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Treatment of VaricellaWho should receive
acyclovir?
Patient population Treatment
Low risk Healthy children 12 yrs None (? 2)
Increased risk gt 12 years, low dose steroids, ASA, pregnancy, chronic skin or lung disease Consider po acyclovir
High risk High dose steroids in past 30d chemo, BMT, babies lt2wks, immunosuppressives, HIV, congenital immunodeficiency IV acyclovir
Initiate therapy within 24 hours of onset of
rash
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Treatment of VaricellaWho should receive
acyclovir?

• IV therapy should also be given to all severely
  ill patients regardless of risks
• Severe disease pneumonitis, hepatitis,
  encephalopathy, DIC, SIADH
• Visceral dissemination is heralded by high fever,
  extensive rash, severe abdominal or back pain

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Why not use acyclovir for everyone?

• Reduces mean lesions from 347 to 294
• Fever defervesces 1 day earlier
• No reduction in 2 bacterial infections,
  pneumonitis or cerebellitis
• Does not reduce household transmission
• S/E headache, nausea, vomiting, rash
• 1 - acute encephalopathy
• ? risk in underlying neuro/renal disorders

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Herpes Zoster

• IV acyclovir for severely immunocompromised
• PO acyclovir for mildly immunocompromised
• Involvement of opthalmic branch of trigeminal
• po acyclovir (IV if lt 2 years of age)
• Optho consult

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What would you do?

• CASE 1
• 3 yo on prednisone (2 mg/kg/d) for nephrotic
  syndrome
• 5 yo sibling just diagnosed with chicken pox
• No prev Varivax, no hx of CP infection

• A) wait and see
• B) oral acyclovir
• C) IV acyclovir
• D) VZIG
• E) call ID!?!

21
What would you do?

• CASE 1
• 3 yo on prednisone (2 mg/kg/d) for nephrotic
  syndrome
• 5 yo sibling just diagnosed with chicken pox
• No prev Varivax, no hx of CP infection

• A) wait and see
• B) oral acyclovir
• C) IV acyclovir
• D) VZIG
• E) call ID!?!

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• CASE 2
• 3 yo on prednisone (2mg/kg/d) for nephrotic
  syndrome
• just diagnosed with chicken pox
• First lesion noted in past 24 hours

• A) wait and see
• B) oral acyclovir
• C) IV acyclovir
• D) VZIG
• E) call ID!?!

What would you do?
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• CASE 2
• 3 yo on prednisone (2mg/kg/d) for nephrotic
  syndrome
• just diagnosed with chicken pox
• First lesion noted in past 24 hours

• A) wait and see
• B) oral acyclovir
• C) IV acyclovir
• D) VZIG
• E) call ID!?!

What would you do?
24

• CASE 3
• 3 yo on flovent (125ug) 2 puffs BID for asthma
  maintenance
• Just diagnosed with chicken pox
• First lesion noted in past 24 hours

• A) wait and see
• B) oral acyclovir
• C) IV acyclovir
• D) VZIG
• E) call ID!?!

What would you do?

• What if this child had received a 5 day course of
  prednisone (2mg/kg) 3 weeks ago?

25

• CASE 3
• 3 yo on flovent (125ug) 2 puffs BID for asthma
  maintenance
• Just diagnosed with chicken pox
• First lesion noted in past 24 hours

• A) wait and see
• B) oral acyclovir
• C) IV acyclovir
• D) VZIG
• E) call ID!?!

What would you do?

• What if this child had received a 5 day course of
  prednisone (2mg/kg) 3 weeks ago?

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Fever and Petechiae

• In prospective cohort studies, 1.8 of children
  with fever gt 38 have petechiae

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CASE

• An 18 mo old child presents with a fever of 38.9
  and is noted by nursing staff to have petechiae.
  Remaining vitals are normal.
• You assess the child to be content and non-toxic
  in appearance. You note mild URTI Sx and
  multiple petechiae on the chest and arms. The
  remainder of your exam is unremarkable.
• How will you proceed?

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• Evaluation of febrile children with petechial
  rashes is there consensus among pediatricians?
  DG Nelson et al. Ped Inf Dis J 1998171135-40.
• ? management is highly variable!

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Why do we worry?

• Fever and petechiae may be a harbinger of
  septicemia
• In the past, many authorities recommended blood
  cultures, lp, and admission for empiric IV
  antibiotics until cultures returned!
• These recommendations where based on published
  series of in-patients with fever and petechiae
  where invasive bacteria where detected at rates
  of up to 17
• Probable selection bias as examined only
  in-patients

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Fever and Petechiae - Etiology
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Who needs further investigation and treatment?

• Toxic, hypotensive, significant lethargy or
  irritability ? Straightforward
• Well appearing child ? not so easy

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Can we be more selective in the ED?

• Incidence of bacteremia in infants and
  children with fever
• and petechiae. Mandl et al. J Pediat
  1997131398-404.
• Prospective cohort study of all children 18yrs
  presenting to the ED with fever and petechiae
  over an 18 mo period
• N 411
• Clinical features recorded general appearance,
  number of petechiae, position of petechiae,
  whether a mechanical cause for petechiae was
  present, purpura
• Labs recorded CBC, cultures, coags, CSF

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Mandl et al Childrens Hosp, Boston
CBC (97) Culture (95) Coags (64) LP (53)
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Mandl et al Childrens Hosp, BostonDiagnostic
predictors for invasive bacteremia
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Mandl et al Childrens Hosp, Boston

• well appearing children with petechiae, but no
  purpura and normal WBC and INR and exceedingly
  unlikely to have serious invasive bacteremia
• No children with petechiae limited to above the
  nipple line had bacteremia

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Do other out-patient studies agree?

• The management of fever and petechiae making
  sense of rash decisions. Brogan Raffles. Arch
  Dis Child 200083506-7.
• Retrospectively and prospectively assessed the
  performance of a 5 factor screening test (N55)
• Factors shock, lethargy, irritability, abnormal
  WBC, elevated CRP
• 5 patients had bacterial sepsis
• Sens 100, Spec 60, PPV 20, NPV 100

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What about petechiae without fever?

• LC Wells et al Arch Dis Child 2001
• Prospective cohort study of children aged lt16 yrs
  presenting to the PED with non-blanching rash
  over a 12 month period
• N233, 15 excluded ? N 218
• 11 bacteremia (all meningococcus)

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Wells et al - Nottingham
Fever gt 37.5 Sensitivity 79, NPV 95
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How should we approach the child with petechiae?

• Clinical Risk Factors
• Toxic appearance, lethargy, irritability
• Petechiae below the nipple line, purpura
• Laboratory Risk Factors
• Abnormal WBC (lt5000, gt15000)
• Elevated INR

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How should we approach the child with petechiae?

• Safe approach Draw CBC, coags and culture (/-
  lp as clinically indicated)
• if no risk factors, may D/C home (may consider a
  4 hour observation period)
• If any positive risk factors, admit for IV
  antibiotics pending culture results
• In a well-appearing child with no purpura and
  petechiae limited to the SVC area (with a
  plausable mechanism ie. vomiting/coughing) an
  argument could be made for not doing bloodwork

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Acute Exfoliating Conditions

• Toxic Epidermal Necrolysis (TEN)
• Incidence 1.3 per million
• Staph Scalded Skin Syndrome (SSSS)
• Incidence unknown

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Toxic Epidermal Necrolysis (TEN)

• A life-threatening, exfoliating disease of the
  skin and mucous membranes
• Hallmark is full-thickness necrosis of the
  epidermis with separation at the dermoepidermal
  junction

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Relationship of TEN to other Exfoliating
Conditions

• Controversal relationship to
• Erythema multiforme minor
• Erythema multiforme major
• Stevens-Johnson syndrome
• Unclear if TEN is distinct entity or severe form
  of EM major or SJS

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A Spectrum of Illness?
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Erythema Multiforme Minor

• Erythematous, target-like lesions, occasionally
  with bullae, symmetrically involving extensor
  surfaces of limbs, palms and soles

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EM Minor

• Cause hypersensitivity reaction (HSV, drugs)
• Course benign, self-limited, usually resolves in
  5 15 days
• Treatment oral antihistamines and topical
  steroids may provide symptomatic relief
• Skin tenderness is not typical and should alert
  to possibility of early TEN

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Erythema Multiforme Major

• Also thought to be a hypersensitivity reaction
• As with EM minor, but with involvement of 2
  mucosal surfaces (precedes rash by 1-2 days)
• Pronounced constitutional symptoms common

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Stevens-Johnson Syndrome

• Is SJS is separate entity from EM major?
• Some feel SJS is a distinct entity as the rash is
  more erythematous and less acral than EM major
• EM major is more commonly triggered by infections
  and SJS by drugs

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SJS vs TEN

• Unsure if on same spectrum or distinct diseases
• Some use BSA to define with
• lt10 SJS
• gt30 TEN
• Histologically SJS has a much higher density cell
  infiltrate (T-lymphocytes) vs TEN (low density
  macrophages and dendrocytes)

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TEN - Pathogenesis

• Majority of cases are likely adverse drug
  reactions (foreign antigen response)
• Mean time from drug to onset 13.6 days
• Higher risk drugs
• NSAIDS 38
• Antibiotics 36 (sulfonamides)
• Anticonvulsants 24 (phenobarb, lamotrigene)
• Corticosteroids 14

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TEN - Clinical Features

• Initial symptoms (1-3 days)
• Fever (100)
• Conjuctivitis (32)
• Pharyngitis (25)
• Pruritis (28)
• Headache, myalgias, arthralgias, vomiting, and
  diarrhea may occur

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TEN - Clinical Features Mucosal Involvement

• Erosive mucosal lesions (1-3 days before skin
  eruption) occur in 97
• Oral (93)
• Ocular (78)
• Genital (63)
• anal

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TEN - Clinical FeaturesSkin Eruption

• Burning / painful skin rash
• Usually begins on face / upper trunk
• Begins as one of
• Diffuse erythema
• Irregular bullae
• Poorly defined dusky or erythematous macules
• Scalp usually spared

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TEN - Disease Progression

• Skin may develop flaccid bullae or may detach
  irregularly
• Skin lesions progress and extend for 3-4 days
  (may progress rapidly over hours)
• Mean BSA involvement 70

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Nikolskis Sign separation of the epidermis
from the dermis by rubbing skin between the
lesions
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Multisystem Involvement

• GI - Mucosal sloughing in esophagus (dysphagia,
  GI bleeding)
• Resp - Tracheal/bronchial erosions
• (Respiratory decompensation)
• Renal - Glomerulonephritis
• Profound fluid and electrolyte disturbances

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Challenges

• Fluid losses
• Infection
• Staph. aureus, Psuedomonas aeruginosa
• Impaired thermoregulation
• Increased energy expenditure

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Therapy - General

• Transfer to a burn center increases survival
• General management principles similar to burn
  management

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? Medical Therapy

• Steroids are controversial
• Appears to be no survival benefit
• No decrease in ocular complications
• Possible increased risk of death from sepsis
• ImmunomodulatorsNo clear evidence for
  plasmapheresis, cyclophosphamide or cyclosporin
• IVIG promising (increased survival shown in
  adults)

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Mortality

• Wide range in published case series
• Death most common from systemic infection
• Poor prognostic factors
• Extremes of age
• Increased BSA involvement
• Elevated serum BUN
• Neutropenia

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Staph Scalded Skin Syndrome

• SSSS is a distinct entity but may be difficult to
  distinguish clinically
• Both have rapidly evolving exfoliation and
  conjunctival involvment
• SSSS has no initial target lesions, the erosions
  are more superficial and less weepy,
  oropharyngeal lesions are rare
• Skin biopsy is helpful
• (SSSS intraepidermal separation)

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Staph Scalded Skin Syndrome

• Typically presents in children lt 6 years
• gt 60 occur in children lt 2 years
• Caused by exotoxin-producing staph
• Children lack immunity to toxins and have slower
  renal clearance of toxin

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Staph Scalded Skin Syndrome

• Abrupt onset fever, skin tenderness, rash
• Often first seen as erythema and crusting around
  the mouth
• Rash spreads down the body
• Bullae formation and desquamation follow

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Staph Scalded Skin Syndrome

• Usually penicillin resistant strains
• Treat with penicillinase-resistant penicillin
  (IV)
• Steroids are contraindicated
• 4 mortality (mostly newborns)