Stevens-Johnson syndrome and toxic epidermal necrolysis

Background

  • SJS and TEN exist on a spectrum of disease
    • SJS involves <10% of BSA
    • TEN involves >30% of BSA
  • Dermatologic emergency

Causes

  • Drugs
    • The most common cause overall[1]
    • Many have been linked. Common offensive agents include: sulfa, quinolones, PCN, ASA, acetaminophen, carbamazepine, NSAIDs, phenytoin, corticosteroids, immunizations
    • High dose or rapid loading of allopurinol[2], lamotrigine[3]
  • Malignancy - lymphoma, brain tumor treated with radiotherapy and antiepileptics[4]
  • Idiopathic
  • Immunosuppression - HIV [5]
  • Infectious
  • Autoimmune- SLE[6]

Clinical Features

Stevens–Johnson syndrome
Mucosal lesions with Stevens-Johnson
  • 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

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 [7]

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
0-1 3.2%
2 12.1%
3 35.3%
4 58.3%
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. Horne NS, Narayan AR, Young RM, Frieri M. Toxic epidermal necrolysis in systemic lupus erythematosus. Autoimmun Rev. 2006 Feb. 5(2):160-4. [Medline]
  7. 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.