Stevens-Johnson syndrome and toxic epidermal necrolysis

Background

  • Stevens-Johnoen Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are a spectrum of disease which ultimately results in blistering and peeling of the skin
  • Mucous membranes can also be involved
  • SJS and TEN exist on a spectrum of disease
    • SJS involves <10% of the skin body surface area
    • TEN involves >30% of the skin body surface area
  • SJS and TEN are considered true dermatologic emergencies

Causes

Clinical Features

  • Often have prodrome (fever, URI symptoms, headache, malaise)
  • Macular rash
    • +/- Target lesions
    • Usually starts centrally, spreads peripherally, and may become confluent
    • May be painful
    • May have +Nikolsky sign (denude when touched)
  • Mucous membranes can be severely affected
    • Eye involvement can be severe
  • In severe cases, respiratory tract and GI involvement may occur

Differential Diagnosis

Oral rashes and lesions

Evaluation

Work-Up

  • CBC
  • CMP
  • ESR
  • CXR
  • Examine eyes/mucosal surfaces

Evaluation

  • Clinical diagnosis

Table of Severe Drug Rashes

Charateristic DRESS SJS/TEN AGEP Erythroderma
Image
Onset of eruption2-6 weeks1-3 weeks48 hours1-3 weeks
Duration of eruption (weeks)Several1-3<1Several
Fever++++++++++++
Mucocutaneous featuresFacial edema, morbilliform eruption, pustules, exfoliative dermattiis, tense bullae, possible target lesionsBullae, atypical target lesions, mucocutaneous erosionsFacial edema, pustules, tense bullae, possible target lesions, possibl emucosal involvementErythematous plaques and edema affecting >90% of total skin surface with or without diffuse exfoliation
Lymph node enlargement+++-++
NeutrophilsElevatedDecreasedVery elevatedElevated
EosinophilsVery elevatedNo changeElevatedElevated
Atypical lymphocytes+--+
Hepatitis+++++++-
Other organ involvementInterstitial nephritis, pneumonitis, myocarditis, and thydoiditisTubular nephritis and tracheobronical necrosisPossiblePossible
Histological pattern of skinPerivascular lymphocytcic infiltrateEpidermal necrosisSubcorneal pustulesNonspecific, unless reflecting Sezary syndrome or other lymphoma
Lymph node histologyLymphoid hyperplasia--No, unless reflecting Sezary syndrome or other malignancy
Mortality (%)105-3555-15

Management

  • Removal of inciting cause if identified
  • Fluid replacement - treat shock with IV fluids according to burn protocols
  • Infection control
  • Wound care
  • Use of IVIG, plasmapheresis, and corticosteroids are controversial but may be beneficial
  • Evidence that Etanercept (TNF-alpha antagonist) may decrease time to skin healing and mortality compared to IV prednisolone [8]

Disposition

  • Admit to burn unit or ICU

Prognosis

Validated with SCORTEN mortality assessment:

One point for each of the following assessed within 1st 24 hours of admission:

  • Age >/= 40 years (OR 2.7)
  • Heart Rate >/= 120 beats per minute (OR 2.7)
  • Cancer/Hematologic malignancy (OR 4.4)
  • Body surface area on day 1; >10% (OR2.9)
  • Serum urea level (BUN) >28mg/dL (>10mmol/L) (OR 2.5)
  • Serum bicarbonate <20mmol/L (OR 4.3)
  • Serum glucose > 252mg/dL (>14mmol/L) (OR5.3)

Predicted mortality based on above total:

Score Mortality
0-13.2%
212.1%
335.3%
458.3%
5+90.0%

See Also

References

  1. Mockenhaupt M (2011). "The current understanding of Stevens–Johnson syndrome and toxic epidermal necrolysis". Expert Review of Clinical Immunology 7 (6): 803–15. doi:10.1586/eci.11.66. PMID 22014021
  2. Halevy S, Ghislain PD, Mockenhaupt M, et al. Allopurinol is the most common cause of Stevens-Johnson syndrome and toxic epidermal necrolysis in Europe and Israel. J Am Acad Dermatol. 2008 Jan. 58(1):25-32. [Medline]
  3. Schlienger RG, Shapiro LE, Shear NH. Lamotrigine-induced severe cutaneous adverse reactions. Epilepsia. 1998. 39 Suppl 7:S22-6. [Medline]
  4. Medscape: Stevens-Johnson Syndrome
  5. Rotunda A, Hirsch RJ, Scheinfeld N, Weinberg JM. Severe cutaneous reactions associated with the use of human immunodeficiency virus medications. Acta Derm Venereol. 2003. 83(1):1-9. [Medline]
  6. Kunimi Y, Hirata Y, Aihara M, Yamane Y, Ikezawa Z. Statistical analysis of Stevens-Johnson syndrome caused by Mycoplasma pneumonia infection in Japan. Allergol Int. 2011;60(4):525-532. doi:10.2332/allergolint.11-OA-0309
  7. Horne NS, Narayan AR, Young RM, Frieri M. Toxic epidermal necrolysis in systemic lupus erythematosus. Autoimmun Rev. 2006 Feb. 5(2):160-4. [Medline]
  8. Wang, C.-W., Yang, L.-Y., Chen, C.-B., Ho, H.-C., Hung, S.-I., Yang, C.-H., … and the Taiwan Severe Cutaneous Adverse Reaction (TSCAR) Consortium. (2018). Randomized, controlled trial of TNF-α antagonist in CTL-mediated severe cutaneous adverse reactions. The Journal of Clinical Investigation, 128(3), 985–996.
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