Erythema Multiforme in Children

Get CMEEM MinorParents pay a lot of attention to the skin of their child and, certainly, the skin exam is a valuable tool for the vigilant clinician (ex, Cap Refill, Petechiae below Nipple Line, Leukemia, RMSF). That being said, the majority of pediatric rashes (See Peds Rash) that present to the ED are often met with a combination of disdain and fear: “This looks like nothing, but is it something?” or “This looks awful, but is it a big deal?” One of the concerning characteristics is the target lesion, but not all target lesions are created equal. Let us take a minute to digest a morsel on Erythema Multiforme (EM).

 

Erythema Multiforme: Basics

  • Erythema multiforme is the skin manifestations of an acute immune-mediated reaction.
  • The immune-mediated reaction is often triggered by:
    • Viral infection – Majority of cases.
    • HSV infection
    • Mycoplasma pneumonaie
    • Medications 
      • In children, medications are more closely related to SJS and TEN.
      • Adults have a stronger association of EM with medications.
    • Immunization [Read, 2015]
  • Erythema multiforme is typically self-limited.
  • Often it is considered to be on the spectrum that includes Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN), but…
    • Current classification distinguish EM from this specific spectrum. [Bastuji-Garin, 1993]
    • Erythema Multiforme differs from SJS, SJS-TEN Overlap, and TEN in: [Auquier-Dunant, 2002]
      • Demographic characteristics
      • Risk factors
        • It is not associated with HIV infection, cancer, or collagen vascular diseases.
        • It has less of an association with medications.

 

Erythema Multiforme: Mimics

  • Erythema multiforme in children is often misdiagnosed. [Read, 2015; Léauté-Labrèze, 2000]
  • Target lesions are NOT pathognomonic for erythema multiforme. [Read, 2015]
  • There are several other conditions that need to be considered when evaluating target lesions:
    • Serum Sickness
    • Kawasaki Disease
    • HSP
    • Lupus Ertyhematosus
    • Urticaria multiforme
      • Often mistaken for erythema multiforme. [Read, 2015]
        • Acute urticaria that are annular and polycyclic wheals
        • Have central clearing and ecchymotic centers
        • Typically start as small macules or papule and then evolve to various sizes.
        • Individual lesions fade within 24 hours.
      • NOT fixed to the extremity distribution like EM is.
        • Involves trunk, face, and extremities.
      • Patient may also have pruritus, angioedema, and/or dermatographism (which is pretty interesting to see).

 

Erythema Multiforme: Diagnosis

  • The classification criteria for EM are based on Bastuji-Garin, 1993.
  • When observing lesions, consider location and document what you see… don’t just say “target lesions”… make note of all characteristics when able (something I am terrible at).
    • EM Minor
      • Epidermal detachment < 10% BSA
      • Acrally distributed lesions (acral = extremities, ears, peripheral parts)
        • Can be typical or raised atypical or combination of lesions
        • Typical Target Lesions = <3cm diameter, symmetric, round, well-defined border, and 3 concentric color zones
        • RAISED Atypical Target Lesions = <3 cm diameter, round, poorly defined border, only 2 concentric color zones.
      • No mucosal involvement
        • EM Major is the same as EM minor but has one or more mucosal surface involved.
    • SJS and TEN
      •  Lesions are different:
        • Flat, atypical target lesions or
        • Widespread macules
      • Amount of Epidermal detachment determines classification
        • SJS has <10% BSA
        • SJS/TEN Overlap has 10% – 30% BSA
        • TEN has >30% BSA

 

Erythema Multiforme: Management

  • Treatment is supportive.
  • Symptomatic care with antipyretics and antihistamines can help.
    • There have been reported concerns that NSAIDs may worsen the condition. [Dore, 2007]
  • If there is a clear association with a medication, cessation of that is warranted.
  • Majority of children with erythema multiforme are able to be discharged and managed as outpatients. [Read, 2015]

 

Moral of the Morsel

  • Distribution matters. If it involves more than the extremities, think twice about calling it erythema multiforme.
  • Not all target lesions are the same. Flat, atypical targets are not consistent with EM.
  • Think about urticarial multiforme. Just another odd condition to keep in mind to ensure we are not misclassifying illnesses.
  • Think worse first! Look for the mucosal membrane involvement closely!

 

References

Read J1, Keijzers GB. Pediatric Erythema Multiforme in the Emergency Department: More Than “Just a Rash”. Pediatr Emerg Care. 2015 Nov 9. PMID: 26555305. [PubMed] [Read by QxMD]

Keller N1, Gilad O1, Marom D1, Marcus N1, Garty BZ1. Nonbullous Erythema Multiforme in Hospitalized Children: A 10-Year Survey. Pediatr Dermatol. 2015 Sep-Oct;32(5):701-3. PMID: 26223537. [PubMed] [Read by QxMD]

Moreau JF1, Watson RS, Hartman ME, Linde-Zwirble WT, Ferris LK. Epidemiology of ophthalmologic disease associated with erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis in hospitalized children in the United States. Pediatr Dermatol. 2014 Mar-Apr;31(2):163-8. PMID: 23679157. [PubMed] [Read by QxMD]

Emer JJ1, Bernardo SG, Kovalerchik O, Ahmad M. Urticaria multiforme. J Clin Aesthet Dermatol. 2013 Mar;6(3):34-9. PMID: 23556035. [PubMed] [Read by QxMD]

Dore J1, Salisbury RE. Morbidity and mortality of mucocutaneous diseases in the pediatric population at a tertiary care center. J Burn Care Res. 2007 Nov-Dec;28(6):865-70. PMID: 17925657. [PubMed] [Read by QxMD]

Forman R1, Koren G, Shear NH. Erythema multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis in children: a review of 10 years’ experience. Drug Saf. 2002;25(13):965-72. PMID: 12381216. [PubMed] [Read by QxMD]

Auquier-Dunant A1, Mockenhaupt M, Naldi L, Correia O, Schröder W, Roujeau JC; SCAR Study Group. Severe Cutaneous Adverse Reactions. Correlations between clinical patterns and causes of erythema multiforme majus, Stevens-Johnson syndrome, and toxic epidermal necrolysis: results of an international prospective study. Arch Dermatol. 2002 Aug;138(8):1019-24. PMID: 12164739. [PubMed] [Read by QxMD]

Léauté-Labrèze C1, Lamireau T, Chawki D, Maleville J, Taïeb A. Diagnosis, classification, and management of erythema multiforme and Stevens-Johnson syndrome. Arch Dis Child. 2000 Oct;83(4):347-52. PMID: 10999875. [PubMed] [Read by QxMD]

Kelly JP1, Auquier A, Rzany B, Naldi L, Bastuji-Garin S, Correia O, Shapiro S, Kaufman DW. An international collaborative case-control study of severe cutaneous adverse reactions (SCAR). Design and methods. J Clin Epidemiol. 1995 Sep;48(9):1099-108. PMID: 7636511. [PubMed] [Read by QxMD]

Bastuji-Garin S1, Rzany B, Stern RS, Shear NH, Naldi L, Roujeau JC. Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme. Arch Dermatol. 1993 Jan;129(1):92-6. PMID: 8420497. [PubMed] [Read by QxMD]

Sean Fox

I enjoy taking care of patients and I finding it endlessly rewarding to help train others to do the same. I trained at the Combined Emergency Medicine and Pediatrics residency program at University of Maryland, where I had the tremendous fortune of learning from world renown educators and clinicians. Now I have the unbelievable honor of working with an unbelievably gifted group of practitioners at Carolinas Medical Center. I strive every day to inspire my residents as much as they inspire me.

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2 Responses

  1. Brisson Muia says:

    Good work Sean Fox, your work is exemplary…..

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