Difference between revisions of "Stevens-Johnson syndrome and toxic epidermal necrolysis"

(DDx)
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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]]
*Often have prodrome (fever, URI symptoms, HA, malaise)
+
[[File:SJS.jpg|thumbnail|Mucosal lesions with Stevens-Johnson]]
*Macular rash
+
*Often have prodrome ([[fever]], [[URI]] symptoms, [[headache]], malaise)
 +
*Macular [[rash]]
 
**+/- Target lesions
 
**+/- Target lesions
 
**Usually starts centrally, spreads peripherally, and may become confluent
 
**Usually starts centrally, spreads peripherally, and may become confluent
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*In severe cases, respiratory tract and GI involvement may occur
 
*In severe cases, respiratory tract and GI involvement may occur
  
==Work-Up==
+
==Differential Diagnosis==
 +
*[[Erythema Multiforme]]
 +
*[[Staphylococcal scalded skin syndrome]]
 +
*[[Erythroderma]]
 +
*[[Toxic Shock Syndrome]]
 +
*[[Drug rash]]
 +
*[[Acute generalized exanthematous pustulosis]]
 +
*[[DRESS syndrome]]
 +
 
 +
{{DDX oral rashes and lesions}}
 +
 
 +
==Evaluation==
 +
===Work-Up===
 
*CBC
 
*CBC
*Chem
+
*CMP
 
*ESR
 
*ESR
*CXR
+
*[[CXR]]
 
*Examine eyes/mucosal surfaces
 
*Examine eyes/mucosal surfaces
  
==Differential Diagnosis==
+
===Evaluation===
*[[Erythema Multiforme]]
+
*Clinical diagnosis
*[[Staphlococcal scalded skin syndrome]]
+
{{Table of severe drug reactions}}
*[[Erythroderma]]
 
*[[Toxic Shock Syndrome]]
 
[[*Drug eruption]]
 
  
==Treatment==
+
==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 [[parkland formula|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]]

Latest revision as of 19:17, 17 December 2019

Background

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

Causes

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

Table of Severe Drug Rashes

Charateristic DRESS SJS/TEN AGEP Erythroderma
Image PMC3894017 JFMPC-2-83-g001.png Stevens-johnson-syndrome.jpg Acute generalized exanthematous pustulosis.png Red (burning) Skin Syndrome - Feet Collage.jpg
Onset of eruption 2-6 weeks 1-3 weeks 48 hours 1-3 weeks
Duration of eruption (weeks) Several 1-3 <1 Several
Fever +++ +++ +++ +++
Mucocutaneous features Facial edema, morbilliform eruption, pustules, exfoliative dermattiis, tense bullae, possible target lesions Bullae, atypical target lesions, mucocutaneous erosions Facial edema, pustules, tense bullae, possible target lesions, possibl emucosal involvement Erythematous plaques and edema affecting >90% of total skin surface with or without diffuse exfoliation
Lymph node enlargement +++ - + +
Neutrophils Elevated Decreased Very elevated Elevated
Eosinophils Very elevated No change Elevated Elevated
Atypical lymphocytes + - - +
Hepatitis +++ ++ ++ -
Other organ involvement Interstitial nephritis, pneumonitis, myocarditis, and thydoiditis Tubular nephritis and tracheobronical necrosis Possible Possible
Histological pattern of skin Perivascular lymphocytcic infiltrate Epidermal necrosis Subcorneal pustules Nonspecific, unless reflecting Sezary syndrome or other lymphoma
Lymph node histology Lymphoid hyperplasia - - No, unless reflecting Sezary syndrome or other malignancy
Mortality (%) 10 5-35 5 5-15

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 [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.