Stevens-Johnson syndrome and toxic epidermal necrolysis: Difference between revisions

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==Background==
==Background==
*SJS and TEN exist on a spectrum of disease
*SJS and TEN exist on a spectrum of disease
**SJS involves <10% of BSA
**SJS involves <10% of [[BSA]]
**TEN involves >30% of BSA
**TEN involves >30% of [[BSA]]
*Dermatologic emergency
*Dermatologic emergency
*Causes:
 
**Drugs - many. Common offensive agents include: quinolones, sulfa, PCN, ASA, acetaminophen, carbamazepine, NSAIDs, phenytoin, corticosteroids, immunizations
===Causes===
**Malignancy - lymphoma
*Drugs
**Idiopathic
**The most common cause overall<ref>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</ref>
**Infectious
**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<ref>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]</ref>, lamotrigine<ref>Schlienger RG, Shapiro LE, Shear NH. Lamotrigine-induced severe cutaneous adverse reactions. Epilepsia. 1998. 39 Suppl 7:S22-6. [Medline]</ref>
*Malignancy - lymphoma, brain tumor treated with radiotherapy and antiepileptics<ref>[http://emedicine.medscape.com/article/1197450-overview#a4 Medscape: Stevens-Johnson Syndrome]</ref>
*Idiopathic
*Immunosuppression - [[HIV]] <ref>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]</ref>
*Infectious
*Autoimmune- SLE<ref>Horne NS, Narayan AR, Young RM, Frieri M. Toxic epidermal necrolysis in systemic lupus erythematosus. Autoimmun Rev. 2006 Feb. 5(2):160-4. [Medline]</ref>


==Clinical Features==
==Clinical Features==
[[File:Stevens-johnson-syndrome.jpg|thumbnail|Stevens–Johnson syndrome]]
[[File:Stevens-johnson-syndrome.jpg|thumbnail|Stevens–Johnson syndrome]]
[[File:SJS.jpg|thumbnail|Mucosal lesions with Stevens-Johnson]]
[[File:SJS.jpg|thumbnail|Mucosal lesions with Stevens-Johnson]]
*Often have prodrome (fever, URI symptoms, HA, malaise)
*Often have prodrome (fever, URI symptoms, headache, malaise)
*Macular rash
*Macular rash
**+/- Target lesions
**+/- Target lesions
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**Eye involvement can be severe
**Eye involvement can be severe
*In severe cases, respiratory tract and GI involvement may occur
*In severe cases, respiratory tract and GI involvement may occur
==Work-Up==
*CBC
*Chem
*ESR
*CXR
*Examine eyes/mucosal surfaces


==Differential Diagnosis==
==Differential Diagnosis==
*[[Erythema Multiforme]]
*[[Erythema Multiforme]]
*[[Staphlococcal scalded skin syndrome]]
*[[Staphylococcal scalded skin syndrome]]
*[[Erythroderma]]
*[[Erythroderma]]
*[[Toxic Shock Syndrome]]
*[[Toxic Shock Syndrome]]
*[[Drug eruption]]
*[[Drug eruption]]
*[[Acute generalized exanthematous pustulosis]]
*[[DRESS syndrome]]
{{DDX oral rashes and lesions}}
==Evaluation==
===Work-Up===
*CBC
*CMP
*ESR
*[[CXR]]
*Examine eyes/mucosal surfaces


==Treatment==
===Evaluation===
*Clinical diagnosis
 
==Management==
*Removal of inciting cause if identified
*Removal of inciting cause if identified
*Fluid replacement - treat shock w/ IV fluids according to burn protocols
*Fluid replacement - treat shock with IV fluids according to burn protocols
*Infection control
*Infection control
*Wound care
*Wound care
*Use of IVIG, plasmapheresis, and corticosteroids are controversial but may be beneficial
*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 <ref>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.</ref>


== Disposition ==
==Disposition==
*Admit to burn unit or ICU
*Admit to burn unit or ICU


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


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


'''Predicted mortality based on above total:'''  
'''Predicted mortality based on above total:'''  


Score 0-1 (3.2%)
{| {{table}}
 
| align="center" style="background:#f0f0f0;"|'''Score'''
2&nbsp; (12.1%)
| align="center" style="background:#f0f0f0;"|'''Mortality'''
 
|-
3&nbsp; (35.3%)
| 0-1||3.2%
 
|-
4&nbsp; (58.3%)<br>
| 2||12.1%
|-
| 3||35.3%
|-
| 4||58.3%
|-
| 5+||90.0%
|}


5+&nbsp; (90.0%)
==See Also==
*[[Rashes]]


== Source ==
==References==
*Tintinalli
<References/>
*Rosen's
*UpToDate
*Sylvie Bastuji-Garin, Nathalie Fouchard*, M Bertocchi*, Jean-Claude Roujeau*, Jean Revuz* and Pierre Wolkenstein. SCORTEN: A Severity-of-Illness Score for Toxic Epidermal Necrolysis. Journal of Investigative Dermatology (2000) 115, 149–153<br>
*Rob Cartotto, MD, FRCS(C), Mike Mayich, BSc, Duncan Nickerson, MD, FRCS(C), Manuel Gomez, MD, MSc. SCORTEN Accurately Predicts Mortality Among Toxic Epidermal Necrolysis Patients Treated in a Burn Center. J Burn Care Res 2008;29:141–146<br>


[[Category:Derm]]
[[Category:Dermatology]]
[[Category:Pharmacology]]
[[Category:Critical Care]]

Revision as of 07:39, 12 February 2019

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.