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Peds Derm 101 Cases Itching to be Solved May 22, 2015 HILTON HEAD ISLAND, SC Emily Berger, MD Helen T. Shin, MD Pediatric Dermatology Joseph M. Sanzari Children’s Hospital Hackensack University Medical Center

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Page 1: Peds Derm 101 HH Handout

Peds Derm 101 Cases Itching to be Solved

May 22, 2015 HILTON HEAD ISLAND, SC

Emily  Berger,  MD  Helen  T.  Shin,  MD  Pediatric  Dermatology  Joseph  M.  Sanzari  Children’s  Hospital  Hackensack  University  Medical  Center    

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“Eczema  Coxsackium”  Disseminated  coxsackievirus  A6  infecJng  eczematous  areas  Perioral,  extremiJes,  trunk  +  hand,  foot,  buMocks  Coxsackievirus  A6  (CV-­‐A6)  emerging  pathogenà  atypical  hand,  foot,  

 mouth  disease  Reported  in  US  and  abroad  Fever,  systemic  symptoms  not  uncommon  In  kids  with  AD  à  widespread  papular  or  vesicular  erupJon  

 mimicking  eczema  herpeJcum,  chickenpox,  etc.  DifferenJated  from  eczema  herpeJcum:  more  generalized,  more  

 discrete  (vs  clustered)  lesions,  less  well-­‐circumscribed  lesions  Other  presentaJons  of  CV-­‐A6:  GCS-­‐like,  purpuric,  delayed  

 onychomadesis  (nail  shedding)  

Lynch  MD,  et  al.  Disseminated  coxsackievirus  A6  affecJng  children  with  atopic  dermaJJs.  Clin  Exp  Dermatol.  2015  Feb  10.  doi:  10.1111/ced.12574.  [Epub  ahead  of  print]  Mathes  EF,  et  al.    “Eczema  coxsackium”  and  unusual  cutaneous  findings  in  an  enterovirus  outbreak.  Pediatrics.  2013;132:e149-­‐7.  

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Peds Derm 101 Erythema infectiosum

²  Parvovirus B19 ²  Asymptomatic infection ²  Exanthematous disorders

²  Erythema infectiosum (fifth disease) ²  Papular-purpuric gloves and socks syndrome ²  Asymmetric periflexural exanthem ²  ‘Bathing trunk’ exanthem ²  Petechial exanthems

²  Other disorders ²  Arthritis ²  Transient aplastic crises ²  Chronic anemia ²  Refractory anemia following solid organ or stem cell transplantation ²  Fetal hydrops ²  Vasculitis ²  Neurologic disease ²  Rheumatologic disease

Paller  AS,  Mancini  AJ,  eds.  Hurwitz  Clinical  Pediatric  Dermatology.  4th  ed.  Elsevier  2011.  

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Gianoh-­‐CrosJ  Syndrome  

Described  in  1955-­‐1956  Edematous,  erythematous,  monomorphous  papular  (or  papulovesicular)  erupJon  symmetrically  distributed  on  the  face,  buMocks,  and  extensor  extremiJes  Children  ages  1-­‐6  years  EJology  –  uncommonly  HepaJJs  B  as  iniJally  described:  EBV,  CMV,  Coxsackie,  adenovirus,  RSV,  parainfluenza  virus,  parvovirus  B19,  rotavirus,  HHV-­‐6,  etc  VaccinaJons  8-­‐12  weeks  course  Management  is  supporJve  

   

Paller  AS,  Mancini  AJ,  eds.  Hurwitz  Clinical  Pediatric  Dermatology.  4th  ed.  Elsevier  2011.  

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Berger  EM,  Orlow  SJ,  Patel  RR,  Schaffer  JV.  Experience  With  Molluscum  Contagiosum  and  Associated  Inflammatory  ReacFons  in  a  Pediatric  Dermatology  PracFce:  The  Bump  That  Rashes.  Arch  Dermatol.  2012;148:1257-­‐1264.  

Gianoh-­‐CrosJ  Syndrome-­‐Like  (ID)  ReacJon  to  Inflamed  Molluscum  

Immune  response  to  molluscum  contagiosum    virus  (MCV)    

Under-­‐recognized  (~5%  of  paJents  in  large    series  of  MCV)  

Good  prognosJc  sign  Average  Jme  unJl  resoluJon  of  MCV=  2  months  

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“Id  ReacJon”  “AutoeczemaJzaJon”  ReacJon  to  nickel  (dermaJJs  under  umbilicus),    Jnea  capiJs,  etc  

Treat  underlying  condiJon  SymptomaJc  management:  topical  corJcosteroids,    oral  anJhistamines,  PO  corJcosteroids  if  severe    

Treatment  of  Jnea  capiJs:  6-­‐8  week  course  of    griseofulvin  microsize  20-­‐25  mg/kg/day  divided    BID,  give  with  faMy  food,  emphasize  fomite    removal  

 Paller  AS,  Mancini  AJ,  eds.  Hurwitz  Clinical  Pediatric  Dermatology.  4th  ed.  Elsevier  2011.  

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Molluscum  DermaJJs  

More  common  if  underlying  atopic  dermaJJs  (AD)  AD  is  an  indicaJon  to  favor  molluscum  treatment  Topical  corJcosteroids  for  dermaJJs  management  

 

Berger  EM,  Orlow  SJ,  Patel  RR,  Schaffer  JV.  Experience  With  Molluscum  Contagiosum  and  Associated  Inflammatory  ReacJons  in  a  Pediatric  Dermatology  PracJce:  The  Bump  That  Rashes.  Arch  Dermatol.  2012;148:1257-­‐1264.  

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InfanJle  Eczema  “Headlight  sign”  May  be  super-­‐infected  with  S.  aureus,  but  NOT  primary  process    

Topical  vs  oral  anJbioJc  appropriate  in  many  cases    Irritant  contact  dermaJJs  component  (drool,    foods,  etc.)    

Treatment:  Low-­‐  to  mid-­‐potency  topical    corJcosteroid,  barrier  cream/ointment  for    irritant  component  

Paller  AS,  Mancini  AJ,  eds.  Hurwitz  Clinical  Pediatric  Dermatology.  4th  ed.  Elsevier  2011.  

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“Eczema  HerpeJcum”    

AKA  “Kaposi’s  varicelliform  erupJon”  Skin  superinfecJon  w/  HSV    In  paJents  w/  preexisJng  dermatosis  (most    commonly  AD)  

Clusters  of  umbilicated  vesicles,  monomorphous    “punched  out”  erosions  

Commonly  impeJginized  If  periocular  involvement,  STAT  ophtho  eval  Associated  systemic  symptoms  Treatment:  anJviral  (IV  or  PO)  If  frequent  recurrences,  prophylacJc  PO  anJviral    Jen  M,  Chang  MW.  Eczema  herpeJcum  and  eczema  vaccinatum  in  children.  Pediatric  Annals.  2010;39:  658-­‐64  Luca  NJ,  Lara-­‐Corrales  I,  Pope  E.  Eczema  herpeJcum  in  children:  clinical  features  and  factors  predicJve  of  hospitalizaJon.  J  Pediatr.  2012;161:671-­‐5.  

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Staph  Scalded  Skin  Syndrome  “RiMer’s  disease”  Staph  aureus  phage  II  types  3A,  3C,  55,  71à  ExfoliaJve  toxins  

 A  &  B  àcleave  desmoglein  1  Pediatric  cases:  premature  infants,  children  <6  Perioral  area  =  diagnosJc  clue    Prodromeà  Erythema  then  exfoliaJon  @  flexures  à  Skin  

 sloughing  lasts  3-­‐5  daysà  Resolves  in  1  to  2  weeks  Culture  for  a  source:  skin,  orifices    Sterile  bullae    Treatment:  Beta-­‐lactamase  resistant  agents,  clindamycin  (vs.  

 toxin  producJon)  at  home  or  inpaJent    

  Bolognia,  et  al,  eds.  Dermatology.  2008  Elsevier.      

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“UrJcaria  MulJforme”  aka  Annular  UrJcaria  

Skin  ErupJon:  Annular  and  polycyclic  wheals  with  central  clearing  or  ecchymoJc  centers    N=  18    Clinical  Features:    Pruritus  (94%)  Angioedema  of  hands  and  feet  or  face  (72%)    Dermatographism  (44%)    Fever  (44  %)    Symptoms  suggesJve  of  recent  viral  or  bacterial  illness  (67%)    Recent  anJbioJc  use    (44%)  Recent  ImmunizaJons  (11%)    

 Shah KN, Honig PJ, Yan AC. “Urticaria Multiforme”: A Case Series and Review of Acute Annular Urticarial Hypersensitivity Syndromes in Children. Pediatrics. 2007 May;119(5):e1177-83.

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Shah KN, Honig PJ, Yan AC. “Urticaria Multiforme”: A Case Series and Review of Acute Annular Urticarial Hypersensitivity Syndromes in Children. Pediatrics. 2007 May;119(5):e1177-83.

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Erythema  MulJforme  (EM)  •  90  %  of  cases  precipitated  by  infecJon,  most  commonly  

 HSV  1  or  2  •  Typical  target  lesions:  well-­‐defined,  <3cm  papule  or  

 plaque  with  two  concentric  rings  of  color  change  and    central  zone  with  evidence  of  epidermal  damage  

•  Systemic  symptoms:  fever,  weakness,  arthralgias,    atypical  pneumonia-­‐like  lung  changes  (2/2  EM  or    infecJon)  

•  Abrupt  onset  à  2  weeks  typical  episodeà  resolves    without  sequelae  

•  Recurrence  is  common  in  HSV-­‐related  EM    

Bolognia,  et  al,  eds.  Dermatology.  2008  Elsevier.  

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SJS/TEN  •  Rare,  acute,  potenJally  fatal,  

 mucocutaneous  reacJons  •  Epidermal  detachment,  <10%=  SJS  and  

 >30%  =  TEN  •  KeraJnocyte  apoptosis  triggered  by  up  

 regulaJon  of  keraJnocyte  Fas  ligand    (FasL)  expression  

•  Most  cases  are  in  response  to  drugs:    anJbioJcs  (sulfonamide),    anJconvulsants,  NSAIDs  high    offenders  

•  Mortality  rates  thought  to  be  lower  in    kids  (TEN  ~  50%  mortality  in  adults)  

•  PotenJal  long  term  sequelae  @  skin    and  eyes  

•  SupporJve  management  in  ICU,  burn    unit    

•  IVIG  >  2  g/kg  advocated  

 Finkelstein  Y,  et  al.  Recurrence  and  outcomes  of  Stevens-­‐Johnson  syndrome  and  toxic  epidermal  necrolysis  in  children.  Pediatrics.  2011;128:723-­‐8.  Barron  SJ,  DelVecchio  MT,  Aronoff  SC.  Intravenous  immunoglobulin  in  the  treatment  of  Stevens-­‐Johnson  syndrome  and  toxic  epidermal  necrolysis:  a  meta-­‐analysis  with  meta-­‐regression  of  observaJonal  studies.  Int  J  Dermatol.  2015;54:108-­‐15.  

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Mucosal  Predominant  or  Atypical  SJS  

“Mycoplasma  pneumonia-­‐  induced  rash  and  mucosiJs”  Likely  other  infecJous  culprits  yet  to  be  idenJfied  May  respond  to  systemic  corJcosteroids  alone  or  in  combinaJon  with  IVIG  Unlikely  to  recur  (8%  in  a  systemaJc  review)  

Canavan  TN,  Mathes  EF,  Frieden  I,  Shinkai  K.  Mycoplasma  pneumonia-­‐induced  rash  and  mucosiJs  as  a  syndrome  disJnct  from  Stevens-­‐Johnson  syndrome  and  erythema  mulJforme:  A  systemaJc  review.  J  Am  Acad  Dermatol.  2015:  72:239-­‐45  Ahluwalia  J,  Wan  J,  Lee  DH,  Treat  J,  Yan  AC.  Mycoplasma-­‐Associated  Stevens-­‐Johnson  Syndrome  in  children:  RetrospecJve  review  of  paJents  managed  with  or  without  intravenous  immunoglobulin,  systemic  corJcosteroids,  or  a  combinaJon  of  therapies.  Pediatr  Dermatol.  2014;31:664-­‐9  

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Exanthematous Drug Eruption Etiologic Agents

•  Amoxicillin •  Ampicillin •  Bleomycin •  Captopril •  Carbamazepine •  Chlorpromazine •  Cotrimoxazole

•  Gold •  Nalidixic acid •  Naproxen •  Phenytoin •  Penicillamine •  Piroxicam

Litt J. Drug eruption reference manual. NY: Parthenon Publishing Group; 2000

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Drug Hypersensitivity Syndrome (DRESS)

²  DRESS (drug reaction with eosinophilia and systemic symptoms)

²  1 in 1,000 to 1 in 10,000 anticonvulsant exposures ²  Clinical features

–  Triad - fever, rash, systemic involvement –  Onset 7 to 28 days, first exposure –  Periorbital and facial edema –  Generalized lymphadenopathy –  Cutaneous eruption

Husain Z et al. DRESS syndrome Part I. Clinical Perspectives. J Am Acad Dermatol 2013;68:693.e1-14 Husain Z et al. DRESS syndrome Part II. Management and Therapeutics. J Am Acad Dermatol 2013;68:709.e1-9

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Drug Hypersensitivity Syndrome (DRESS)

²  Hepatitis ² Hematologic abnormalities ² Renal damage ² Pulmonary, cardiac, CNS ² Lymphomatoid changes

²  Benign lymphoid hyperplasia ²  Pseudolymphoma

² Thyroiditis - 2 months

Husain Z et al. DRESS syndrome Part I. Clinical Perspectives. J Am Acad Dermatol 2013;68:693.e1-14 Husain Z et al. DRESS syndrome Part II. Management and Therapeutics. J Am Acad Dermatol 2013;68:709.e1-9

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Drug Hypersensitivity Syndrome Management

²  Discontinue medication ²  LFTs, CBC w/ smear, UA, creatinine ²  Chest x-ray ²  Skin biopsy ²  Reassess at 3 weeks, 2-3 months ²  Supportive therapy ²  1.0-2.0 mg/kg prednisone - slow taper ²  Council family members

Husain Z et al. DRESS syndrome Part I. Clinical Perspectives. J Am Acad Dermatol 2013;68:693.e1-14 Husain Z et al. DRESS syndrome Part II. Management and Therapeutics. J Am Acad Dermatol 2013;68:709.e1-9

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Pityriasis  Rosea  Self  limited  exanthem  “Herald  patch”à  few  days  to  3  weeksà  crops  of  ovoid  

 papules  and  plaques  with  collareMes  of  scaleà  6  wks  +    for  resoluJon  

¼  kids  are  itchy  CharacterisJc  “Christmas  tree”  paMern  on  trunk  and  

 extremiJes  Variants:  “inverse”  paMern  @  skin  folds,  face;  round  papules  (young  children,  skin  of  color);  vesicular;  pustular;  urJcarial;  hemorrhagic  

Treatment  is  supporJve  Macrolide  anJbioJcs  ineffecJve  Newer  RCT  for  acyclovir  

Paller  AS,  Mancini  AJ,  eds.  Hurwitz  Clinical  Pediatric  Dermatology.  4th  ed.  Elsevier  2011.  Ganguly  S.  A  Randomized,  Double-­‐blind,  Placebo-­‐controlled  Study  of  Efficacy  of  Oral  Acyclovir  in  the  Treatment  of  Pityriasis  Rosea.    J  Clin  DiagnosJc  Research.    2014;8:  YC01-­‐04.    

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Pediatric  Psoriasis  At  least  1/3  of  paJents  with  psoriasis  recall  having  

 psoriasis  as  children  GuMate  =  “droplike”  

2-­‐6  mm  round  or  oval  papules,  symmetrically  distributed  Preceding  group  A  streptococcal  infecJon  predicts  

 guMate  morphology  but  not  psoriasis  severity    Progression  to  psoriasis  vulgaris  ~40%  of  kids  with  

 guMate  psoriasis    AnJbioJcs  needed  to  treat  GAS  but  not  helpful  for  

 psoriasis  Case  reports  of  tonsillectomy  for  guMate  psoriasis  

 Mercy,  et  al.  Clinical  manifestaJons  of  pediatric  psoriasis:  Results  of  a  mulJcenter  study  in  the  United  States.  Pediatr  Dermatol.  2013;30:424-­‐8.  Wu  W,  Debbaneh  M,  Moslehi  H,  Koo  J,  Liao  W.  Tonsillectoy  as  a  treatment  for  psoriasis:  A  Review.  J  Dermatolog  Treat.  2014;25:482-­‐6.  

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Persistent  Arthropod  Bite  ReacJon  

“Papular  urJcaria”  HypersensiJvity  reacJon  Recurrent  episodes  if  recurring  bites  SomeJmes  requires  skin  biopsy  (to  help  convince  the  family)  Treatment:  Eliminate  source  

SupporJve  management:  anJhistamines,  topical  corJcosteroids  

Summer’s  coming!    Long-­‐sleeved  clothing    Insect  repellent  

Paller  AS,  Mancini  AJ,  eds.  Hurwitz  Clinical  Pediatric  Dermatology.  4th  ed.  Elsevier  2011.  

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Scabies  O{en  nodular  in  babies:  vigorous  hypersensiJvity  response  on  trunk,  axillae,  diaper  area  5%  permethrin  cream,  technically  not  for  use  under  2  months  of  age  Treatment  repeated  in  1  wk  Treat  scalp  in  infants  Signs  may  not  clear  for  2-­‐6+  weeks  a{er  treatment  Most  common  reason  for  treatment  failure  is  not  treaJng  close  contacts!  

     

Paller  AS,  Mancini  AJ,  eds.  Hurwitz  Clinical  Pediatric  Dermatology.  4th  ed.  Elsevier  2011.  

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Neonatal  Varicella  Disseminated,  erythematous  papules,  vesicles,  erosions  Maternal  infecJon  with  Varicella  Zoster  Virus  (VZV),  ie  

 chickenpox,  during  last  wks  of  pregnancy  Mild  disease:  disease  onset  in  mother  >=  5  days  before  

 delivery  or  in  newborn  during  first  4  days  of  life  More  severe  disease:  disease  in  mother  <5  days  before  to  2  days  a{er  delivery  or  in  newborn  @  5-­‐10  days  of  life  Severe  disease:  Pneumonia,  hepaJJs,    meningoencephaliJs,  coagulopathy,  mortality  Treatment:  VZIG,  IV  acyclovir  (especially  if  chickenpox  in  

 the  mother  within  5  days  before  or  2  days  a{er    delivery)  

Paller  AS,  Mancini  AJ,  eds.  Hurwitz  Clinical  Pediatric  Dermatology.  4th  ed.  Elsevier  2011.  

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