Stevens-Johnson syndrome and toxic epidermal necrolysis: Difference between revisions
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==Background== | ==Background== | ||
*SJS and TEN | *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 | |||
== | ===Causes=== | ||
*Often have prodrome (fever, URI symptoms, | *Drugs | ||
* | **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> | ||
**Target lesions | **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== | |||
[[File:Stevens-johnson-syndrome.jpg|thumbnail|Stevens–Johnson syndrome]] | |||
[[File:SJS.jpg|thumbnail|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 | *Mucous membranes can be severely affected | ||
**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== | ==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 | ||
* | *CMP | ||
*ESR | *ESR | ||
*CXR | *[[CXR]] | ||
== | *Examine eyes/mucosal surfaces | ||
===Evaluation=== | |||
*Clinical diagnosis | |||
{{Table of severe drug reactions}} | |||
==Management== | |||
*Removal of inciting cause if identified | *Removal of inciting cause if identified | ||
* | *[[Fluid replacement]] - treat [[shock]] with IV fluids according to [[parkland formula|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 <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 | ||
== | ==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:''' | |||
{| {{table}} | |||
| align="center" style="background:#f0f0f0;"|'''Score''' | |||
| align="center" style="background:#f0f0f0;"|'''Mortality''' | |||
|- | |||
| 0-1||3.2% | |||
|- | |||
| 2||12.1% | |||
|- | |||
| 3||35.3% | |||
|- | |||
| 4||58.3% | |||
|- | |||
| 5+||90.0% | |||
|} | |||
==See Also== | |||
*[[Rashes]] | |||
==References== | |||
<References/> | |||
[[Category: | [[Category:Dermatology]] | ||
[[Category:Pharmacology]] | |||
[[Category:Critical Care]] |
Revision as of 19:17, 17 December 2019
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 rash
- 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
Table of Severe Drug Rashes
Charateristic | DRESS | SJS/TEN | AGEP | Erythroderma |
Image | ||||
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
- ↑ 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.