Stevens-Johnson syndrome and toxic epidermal necrolysis
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Background
- SJS and TEN exist on a spectrum of disease
- 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
- 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
- Erythema Multiforme
- Staphylococcal scalded skin syndrome
- Erythroderma
- Toxic Shock Syndrome
- Drug eruption
- Acute generalized exanthematous pustulosis
- DRESS syndrome
Oral rashes and lesions
- Angioedema
- Aphthous stomatitis
- Herpes gingivostomatitis
- Herpes labialis
- Measles (Koplik's spots)
- Perioral dermatitis
- Oral thrush
- Steven Johnson syndrome
- Streptococcal pharyngitis
- Tongue diagnoses
- Vincent's angina
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
- ↑ 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
- ↑ 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]
- ↑ Schlienger RG, Shapiro LE, Shear NH. Lamotrigine-induced severe cutaneous adverse reactions. Epilepsia. 1998. 39 Suppl 7:S22-6. [Medline]
- ↑ Medscape: Stevens-Johnson Syndrome
- ↑ 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]
- ↑ Horne NS, Narayan AR, Young RM, Frieri M. Toxic epidermal necrolysis in systemic lupus erythematosus. Autoimmun Rev. 2006 Feb. 5(2):160-4. [Medline]
- ↑ 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.