Stevens-Johnson syndrome and toxic epidermal necrolysis: Difference between revisions
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==Background== | ==Background== | ||
* Stevens-Johnoen Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are a spectrum of disease which ultimately results in blistering and peeling of the skin | |||
* Mucous membranes can also be involved | |||
*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 the skin [[BSA|body surface area]] | ||
**TEN involves >30% of BSA | **TEN involves >30% of the skin [[BSA|body surface area]] | ||
* | *SJS and TEN are considered true dermatologic emergencies | ||
===Causes=== | ===Causes=== | ||
*Drugs | *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> | **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> | ||
**Many have been linked. Common offensive agents include: sulfa, quinolones, PCN, ASA, acetaminophen, carbamazepine, NSAIDs, phenytoin, corticosteroids, immunizations | **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> | **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> | *Malignancy - [[lymphoma]], [[brain tumor]] treated with radiotherapy and antiepileptics<ref>[http://emedicine.medscape.com/article/1197450-overview#a4 Medscape: Stevens-Johnson Syndrome]</ref> | ||
*Idiopathic | *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> | *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 | *Infectious - [[Mycoplasma pneumoniae]]<ref>Kunimi Y, Hirata Y, Aihara M, Yamane Y, Ikezawa Z. Statistical analysis of Stevens-Johnson syndrome caused by Mycoplasma pneumonia infection in Japan. Allergol Int. 2011;60(4):525-532. doi:10.2332/allergolint.11-OA-0309</ref> | ||
*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> | *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, headache, malaise) | *Often have prodrome ([[fever]], [[URI]] symptoms, [[headache]], malaise) | ||
*Macular rash | *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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==Differential Diagnosis== | ==Differential Diagnosis== | ||
*[[Erythema Multiforme]] | *[[Erythema Multiforme]] | ||
*[[ | *[[Staphylococcal scalded skin syndrome]] | ||
*[[Erythroderma]] | *[[Erythroderma]] | ||
*[[Toxic Shock Syndrome]] | *[[Toxic Shock Syndrome]] | ||
*[[Drug | *[[Drug rash]] | ||
*[[Acute generalized exanthematous pustulosis]] | *[[Acute generalized exanthematous pustulosis]] | ||
*[[DRESS syndrome]] | *[[DRESS syndrome]] | ||
Line 45: | Line 47: | ||
*CMP | *CMP | ||
*ESR | *ESR | ||
*CXR | *[[CXR]] | ||
*Examine eyes/mucosal surfaces | *Examine eyes/mucosal surfaces | ||
===Evaluation=== | ===Evaluation=== | ||
*Clinical diagnosis | *Clinical diagnosis | ||
{{Table of severe drug reactions}} | |||
==Management== | ==Management== | ||
*Removal of inciting cause if identified | *Removal of inciting cause if identified | ||
*Fluid replacement - treat shock with 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== | ||
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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 | *Age >/= 40 years (OR 2.7) | ||
*Heart Rate | *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 | *Body surface area on day 1; >10% (OR2.9) | ||
*Serum urea level (BUN) | *Serum urea level (BUN) >28mg/dL (>10mmol/L) (OR 2.5) | ||
*Serum bicarbonate | *Serum bicarbonate <20mmol/L (OR 4.3) | ||
*Serum glucose | *Serum glucose > 252mg/dL (>14mmol/L) (OR5.3) | ||
'''Predicted mortality based on above total:''' | '''Predicted mortality based on above total:''' |
Revision as of 16:33, 5 January 2021
Background
- Stevens-Johnoen Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are a spectrum of disease which ultimately results in blistering and peeling of the skin
- Mucous membranes can also be involved
- SJS and TEN exist on a spectrum of disease
- SJS involves <10% of the skin body surface area
- TEN involves >30% of the skin body surface area
- SJS and TEN are considered true dermatologic emergencies
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 - Mycoplasma pneumoniae[6]
- Autoimmune - SLE[7]
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 [8]
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]
- ↑ Kunimi Y, Hirata Y, Aihara M, Yamane Y, Ikezawa Z. Statistical analysis of Stevens-Johnson syndrome caused by Mycoplasma pneumonia infection in Japan. Allergol Int. 2011;60(4):525-532. doi:10.2332/allergolint.11-OA-0309
- ↑ 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.