Stevens-Johnson syndrome overview: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Stevens-Johnson syndrome}} | {{Stevens-Johnson syndrome}} | ||
{{CMG}} | {{CMG}}; {{AE}} {{AHS}} | ||
==Overview== | ==Overview== | ||
'''Stevens-Johnson syndrome''' is a life-threatening [[condition]] affecting the [[skin]], in which due to [[cell death]] the [[epidermis]] separates from the [[dermis]]. The syndrome is thought to be a [[hypersensitivity]] complex affecting the skin and the [[mucous membrane]]s. Although the majority of cases are [[idiopathic]], the main class of known causes is medications, followed by infections and (rarely) cancers. | '''Stevens-Johnson syndrome''' is a life-threatening [[condition]] affecting the [[skin]], in which due to [[cell death]] the [[epidermis]] separates from the [[dermis]]. The syndrome is thought to be a [[hypersensitivity]] complex affecting the skin and the [[mucous membrane]]s. Although the majority of cases are [[idiopathic]], the main class of known causes is medications, followed by infections and (rarely) cancers. | ||
==Historical Perspective== | |||
It is named for Dr. Albert Mason Stevens and Dr. Frank Chambliss Johnson, American pediatricians who jointly published a description of the disorder in 1922.<ref>{{WhoNamedIt|synd|1501|Stevens-Johnson syndrome}}</ref><ref>[http://dictionary.reference.com/browse/Stevens-Johnson%20syndrome Stevens-Johnson syndrome - Definitions from Dictionary.com</ref> | |||
==Classification== | |||
There is agreement in the medical literature that [[Stevens-Johnson syndrome]] can be considered a milder form of [[toxic epidermal necrolysis]] (TEN). Stevens-Johnson Syndrome involves less than 10 percent of body surface area while TEN involve more than 30 percent body surface area. However, there is debate whether it falls on a spectrum of disease that includes [[erythema multiforme]] and [[erythema multiforme major]] (also known as erythema multiforme majus).<ref>{{cite journal |author=Carrozzo M, Togliatto M, Gandolfo S |title=[Erythema multiforme. A heterogeneous pathologic phenotype] |journal=Minerva Stomatol |volume=48 |issue=5 |pages=217-26 |year=1999 |pmid=10434539}}</ref><ref>{{cite journal |author=Farthing P, Bagan J, Scully C |title=Mucosal disease series. Number IV. Erythema multiforme |journal=Oral Dis |volume=11 |issue=5 |pages=261-7 |year=2005 |pmid=16120111}}</ref><ref>{{cite journal |author=Bastuji-Garin S, Rzany B, Stern RS, Shear NH, Naldi L, Roujeau JC |title=Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme |journal=Archives of dermatology |volume=129 |issue=1 |pages=92-6 |year=1993 |pmid=8420497 | doi = 10.1001/archderm.129.1.92 <!--Retrieved from CrossRef by DOI bot-->}}</ref><ref>Ogundele O, Silverberg MA. Erythema Multiforme. eMedicine.com. URL: http://www.emedicine.com/emerg/topic173.htm. Accessed on: May 6, 2007.</ref><ref name="pmid121647392">{{cite journal | |||
| author = Auquier-Dunant A, Mockenhaupt M, Naldi L, Correia O, Schröder W, Roujeau JC | |||
| title = Correlations between clinical patterns and causes of erythema multiforme majus, Stevens-Johnson syndrome, and toxic epidermal necrolysis: results of an international prospective study | |||
| journal = Arch Dermatol | |||
| volume = 138 | |||
| issue = 8 | |||
| pages = 1019–24 | |||
| year = 2002 | |||
| month = August | |||
| pmid = 12164739 | |||
| doi = | |||
| url = http://archderm.ama-assn.org/cgi/pmidlookup?view=long&pmid=12164739 | |||
| issn = | |||
}}</ref> | |||
==Pathophysiology== | |||
SJS, like [[toxic epidermal necrolysis]] and [[erythema multiforme]], are characterized by confluent epidermal [[necrosis]] with minimal associated inflammation. The acuity is apparent from the (normal) basket weave-like pattern of the [[stratum corneum]]. The exact pathogenesis of SJS/TEN is not completely understood. However, It is believed that SJS/TEN is immune mediated and the result of a MHC Class-I restricted T-cell mediated cytotoxic reaction to drug antigens in keratinocytes leading to [[apoptosis]]<ref name="pmid8466217">{{cite journal| author=Correia O, Delgado L, Ramos JP, Resende C, Torrinha JA| title=Cutaneous T-cell recruitment in toxic epidermal necrolysis. Further evidence of CD8+ lymphocyte involvement. | journal=Arch Dermatol | year= 1993 | volume= 129 | issue= 4 | pages= 466-8 | pmid=8466217 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8466217 }}</ref>. Drug antigens bind to MHC-I and T cell Receptors (TCR) leading to the clonal proliferation of the drug specific [[Cytotoxic T cell|cytotoxic T cells]]. On the activation of these T cells, various cytotoxic signals, including [[Fas/Fas ligand]] , [[Perforin]]/[[Granzyme B]] , and [[Granulysin]], are known to be responsible for mediating the damages in skin lesions and the subsequent worsening of the disease. | |||
== Causes == | |||
Stevens-Johnson Syndrome is caused by drug reactions in the majority of the cases although it is also seen in certain viral and bacterial infections, and certain malignancies. Most commonly implicated drugs include [[sulfa drugs]], [[Allopurinol]], [[Anti-Epileptics|anti-epileptics]] (phenobarbital, carbamazepine, lamotrigine), [[Antibiotic|antibiotics]] (for example Penicillin), and [[Non-steroidal anti-inflammatory drug|NSAIDs]]. Infections that are associated with SJS may include HIV, HSV, Mumps, Mycoplasma, Mycobacteria, Hepatitis and Streptococcal Infection. | |||
== Risk Factors == | |||
Common risk factors in the development of SJS include [[Immunodeficiency]], [[Human Immunodeficiency Virus (HIV)|HIV]] infection, active malignancy (particularly [[Hematological cancers]]), genetic Predisposition ( HLA-B 1502 and HLA-B 5801 variants in particular) on exposure to anti-epileptic medications or Allopurinol. Other risk factors may include past or family history of SJS/TEN, radiation therapy and rapid use of medications. | |||
==Epidemiology and Demographics== | |||
SJS is a rare condition, with a reported incidence of around 2-7 per 1000,000 people per year and a mean mortality rate of 4.8 percent. It is seen in patients of all age groups but particularly in children and young adults. Females are more commonly affected than males | |||
==Natural History, Complications and Prognosis== | |||
SJS proper (with less than 10% of body surface area involved) has the [[mortality rate]] of around 5%. The risk of death can be estimated using the '''[[SCORTEN scale]]''', which takes a number of prognostic indicators into account. Other outcomes include organ damage and blindness. | |||
==Diagnosis== | |||
===History and Symptoms=== | |||
Stevens-Johnson syndrome (SJS) usually begins with a fever, sore throat, and fatigue, which is misdiagnosed and usually treated with antibiotics. Ulcers and other lesions begin to appear in the [[Mucous membrane|mucous membranes]], almost always in the mouth and lips but also in the genital and anal regions. Those in the mouth are usually extremely painful and reduce the patient's ability to eat or drink. [[Conjunctivitis]] of the eyes occurs in about 30% of children who develop SJS. A rash of round lesions about an inch across arises on the face, trunk, arms and legs, and soles of the feet, but usually not the scalp.<ref name="Tigchelaar">{{cite journal |author=Tigchelaar H | coauthors = Kannikeswaran N ; Kamat D |title=Stevens–Johnson Syndrome: An Intriguing Diagnosis |journal=Consultant for Pediatricians|month=December | year = 2008 |url=http://www.pediatricsconsultantlive.com/display/article/1803329/1403936 }}</ref> | |||
=== Physical Examination === | |||
Common physical examination findings of SJS include rash or redness that is seen as red or reddish purple [[Macule|macules]] that enlarge to form [[bullae]], [[Vesicle|vesicles]] and may rupture later to leave denuded skin underneath. [[Nikolsky sign]] may be positive which shows sloughing of the skin by gentle lateral pressure on the erythematous skin. In addition to skin, mucosal involvement is found mostly in oral mucosa in addition to the nasopharynx and ocular involvement-can be seen as edema, erythema, blistering, sloughing, necrosis and ulceration. In addition to these changes: fever, hypotension, and tachycardia can be seen in patients with SJS | |||
=== Laboratory Findings === | |||
SJS is mainly diagnosed through the clinical skin findings. However, a skin biopsy is helpful in making the definitive diagnosis. Other tests are done to either rule out the superimposed bacterial infection, measure disease severity or detect the causative agent or organism. Tests include CBC, BMP, Blood cultures, ESR, CRP, IL-6, ELISA for virus detection among others<ref name="pmid27738400">{{cite journal| author=Çekiç Ş, Canıtez Y, Sapan N| title=Evaluation of the patients diagnosed with Stevens-Johnson syndrome and toxic epidermal necrolysis: a single center experience. | journal=Turk Pediatric Ars | year= 2016 | volume= 51 | issue= 3 | pages= 152-158 | pmid=27738400 | doi=10.5152/TurkPediatriArs.2016.3836 | pmc=5047364 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27738400 }}</ref>. | |||
=== Chest X-Ray === | |||
There are no x-ray findings associated with SJS. However, an x-ray may be helpful in the diagnosis of pulmonary complications of Stevens-Johnson Syndrome, in which CXR may show interstitial lung infiltrates<ref>{{Cite journal | |||
| author = [[Amanda M.. Oakley]] & [[Karthik Krishnamurthy]] | |||
| title = Stevens Johnson Syndrome (Toxic Epidermal Necrolysis) | |||
| year = 2018 | |||
| month = January | |||
| pmid = 29083827 | |||
}}</ref> | |||
== Treatment == | |||
Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis should be managed in a burn unit, Specialized dermatology or Intensive Care unit. Patient's fluid status, hemodynamic stability, wound surface area and pain should be assessed and managed immediately upon admission. Treatment is initially similar to that of patients with thermal burns, and continued care can only be supportive (e.g. IV fluids) and symptomatic (e.g. analgesic mouth rinse for mouth ulcer); there is no specific drug treatment. Treatment with corticosteroids is controversial since it might aggravate the condition or increase risk of secondary infections. Other agents have been used, including [[cyclophosphamide]] and [[cyclosporine]], among with cyclosporin has exhibited some therapeutic success. Intravenous immunoglobulin (IVIG) treatment has shown some promise in reducing the length of the reaction and improving symptoms. Other common supportive measures include the use of topical pain anesthetics and antiseptics, maintaining a warm environment, and intravenous analgesics. An [[ophthalmologist]] should be consulted immediately, as SJS frequently causes the formation of scar tissue inside the eyelids leading to corneal vascularization and impaired vision, as well as a host of other ocular problems. Also, an extensive physical therapy program ensues after the patient is discharged from the hospital. | |||
==References== | ==References== | ||
Latest revision as of 15:40, 15 September 2018
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anila Hussain, MD [2]
Overview
Stevens-Johnson syndrome is a life-threatening condition affecting the skin, in which due to cell death the epidermis separates from the dermis. The syndrome is thought to be a hypersensitivity complex affecting the skin and the mucous membranes. Although the majority of cases are idiopathic, the main class of known causes is medications, followed by infections and (rarely) cancers.
Historical Perspective
It is named for Dr. Albert Mason Stevens and Dr. Frank Chambliss Johnson, American pediatricians who jointly published a description of the disorder in 1922.[1][2]
Classification
There is agreement in the medical literature that Stevens-Johnson syndrome can be considered a milder form of toxic epidermal necrolysis (TEN). Stevens-Johnson Syndrome involves less than 10 percent of body surface area while TEN involve more than 30 percent body surface area. However, there is debate whether it falls on a spectrum of disease that includes erythema multiforme and erythema multiforme major (also known as erythema multiforme majus).[3][4][5][6][7]
Pathophysiology
SJS, like toxic epidermal necrolysis and erythema multiforme, are characterized by confluent epidermal necrosis with minimal associated inflammation. The acuity is apparent from the (normal) basket weave-like pattern of the stratum corneum. The exact pathogenesis of SJS/TEN is not completely understood. However, It is believed that SJS/TEN is immune mediated and the result of a MHC Class-I restricted T-cell mediated cytotoxic reaction to drug antigens in keratinocytes leading to apoptosis[8]. Drug antigens bind to MHC-I and T cell Receptors (TCR) leading to the clonal proliferation of the drug specific cytotoxic T cells. On the activation of these T cells, various cytotoxic signals, including Fas/Fas ligand , Perforin/Granzyme B , and Granulysin, are known to be responsible for mediating the damages in skin lesions and the subsequent worsening of the disease.
Causes
Stevens-Johnson Syndrome is caused by drug reactions in the majority of the cases although it is also seen in certain viral and bacterial infections, and certain malignancies. Most commonly implicated drugs include sulfa drugs, Allopurinol, anti-epileptics (phenobarbital, carbamazepine, lamotrigine), antibiotics (for example Penicillin), and NSAIDs. Infections that are associated with SJS may include HIV, HSV, Mumps, Mycoplasma, Mycobacteria, Hepatitis and Streptococcal Infection.
Risk Factors
Common risk factors in the development of SJS include Immunodeficiency, HIV infection, active malignancy (particularly Hematological cancers), genetic Predisposition ( HLA-B 1502 and HLA-B 5801 variants in particular) on exposure to anti-epileptic medications or Allopurinol. Other risk factors may include past or family history of SJS/TEN, radiation therapy and rapid use of medications.
Epidemiology and Demographics
SJS is a rare condition, with a reported incidence of around 2-7 per 1000,000 people per year and a mean mortality rate of 4.8 percent. It is seen in patients of all age groups but particularly in children and young adults. Females are more commonly affected than males
Natural History, Complications and Prognosis
SJS proper (with less than 10% of body surface area involved) has the mortality rate of around 5%. The risk of death can be estimated using the SCORTEN scale, which takes a number of prognostic indicators into account. Other outcomes include organ damage and blindness.
Diagnosis
History and Symptoms
Stevens-Johnson syndrome (SJS) usually begins with a fever, sore throat, and fatigue, which is misdiagnosed and usually treated with antibiotics. Ulcers and other lesions begin to appear in the mucous membranes, almost always in the mouth and lips but also in the genital and anal regions. Those in the mouth are usually extremely painful and reduce the patient's ability to eat or drink. Conjunctivitis of the eyes occurs in about 30% of children who develop SJS. A rash of round lesions about an inch across arises on the face, trunk, arms and legs, and soles of the feet, but usually not the scalp.[9]
Physical Examination
Common physical examination findings of SJS include rash or redness that is seen as red or reddish purple macules that enlarge to form bullae, vesicles and may rupture later to leave denuded skin underneath. Nikolsky sign may be positive which shows sloughing of the skin by gentle lateral pressure on the erythematous skin. In addition to skin, mucosal involvement is found mostly in oral mucosa in addition to the nasopharynx and ocular involvement-can be seen as edema, erythema, blistering, sloughing, necrosis and ulceration. In addition to these changes: fever, hypotension, and tachycardia can be seen in patients with SJS
Laboratory Findings
SJS is mainly diagnosed through the clinical skin findings. However, a skin biopsy is helpful in making the definitive diagnosis. Other tests are done to either rule out the superimposed bacterial infection, measure disease severity or detect the causative agent or organism. Tests include CBC, BMP, Blood cultures, ESR, CRP, IL-6, ELISA for virus detection among others[10].
Chest X-Ray
There are no x-ray findings associated with SJS. However, an x-ray may be helpful in the diagnosis of pulmonary complications of Stevens-Johnson Syndrome, in which CXR may show interstitial lung infiltrates[11]
Treatment
Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis should be managed in a burn unit, Specialized dermatology or Intensive Care unit. Patient's fluid status, hemodynamic stability, wound surface area and pain should be assessed and managed immediately upon admission. Treatment is initially similar to that of patients with thermal burns, and continued care can only be supportive (e.g. IV fluids) and symptomatic (e.g. analgesic mouth rinse for mouth ulcer); there is no specific drug treatment. Treatment with corticosteroids is controversial since it might aggravate the condition or increase risk of secondary infections. Other agents have been used, including cyclophosphamide and cyclosporine, among with cyclosporin has exhibited some therapeutic success. Intravenous immunoglobulin (IVIG) treatment has shown some promise in reducing the length of the reaction and improving symptoms. Other common supportive measures include the use of topical pain anesthetics and antiseptics, maintaining a warm environment, and intravenous analgesics. An ophthalmologist should be consulted immediately, as SJS frequently causes the formation of scar tissue inside the eyelids leading to corneal vascularization and impaired vision, as well as a host of other ocular problems. Also, an extensive physical therapy program ensues after the patient is discharged from the hospital.
References
- ↑ Template:WhoNamedIt
- ↑ [http://dictionary.reference.com/browse/Stevens-Johnson%20syndrome Stevens-Johnson syndrome - Definitions from Dictionary.com
- ↑ Carrozzo M, Togliatto M, Gandolfo S (1999). "[Erythema multiforme. A heterogeneous pathologic phenotype]". Minerva Stomatol. 48 (5): 217–26. PMID 10434539.
- ↑ Farthing P, Bagan J, Scully C (2005). "Mucosal disease series. Number IV. Erythema multiforme". Oral Dis. 11 (5): 261–7. PMID 16120111.
- ↑ Bastuji-Garin S, Rzany B, Stern RS, Shear NH, Naldi L, Roujeau JC (1993). "Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme". Archives of dermatology. 129 (1): 92–6. doi:10.1001/archderm.129.1.92. PMID 8420497.
- ↑ Ogundele O, Silverberg MA. Erythema Multiforme. eMedicine.com. URL: http://www.emedicine.com/emerg/topic173.htm. Accessed on: May 6, 2007.
- ↑ Auquier-Dunant A, Mockenhaupt M, Naldi L, Correia O, Schröder W, Roujeau JC (2002). "Correlations between clinical patterns and causes of erythema multiforme majus, Stevens-Johnson syndrome, and toxic epidermal necrolysis: results of an international prospective study". Arch Dermatol. 138 (8): 1019–24. PMID 12164739. Unknown parameter
|month=ignored (help) - ↑ Correia O, Delgado L, Ramos JP, Resende C, Torrinha JA (1993). "Cutaneous T-cell recruitment in toxic epidermal necrolysis. Further evidence of CD8+ lymphocyte involvement". Arch Dermatol. 129 (4): 466–8. PMID 8466217.
- ↑ Tigchelaar H (2008). "Stevens–Johnson Syndrome: An Intriguing Diagnosis". Consultant for Pediatricians. Unknown parameter
|coauthors=ignored (help); Unknown parameter|month=ignored (help) - ↑ Çekiç Ş, Canıtez Y, Sapan N (2016). "Evaluation of the patients diagnosed with Stevens-Johnson syndrome and toxic epidermal necrolysis: a single center experience". Turk Pediatric Ars. 51 (3): 152–158. doi:10.5152/TurkPediatriArs.2016.3836. PMC 5047364. PMID 27738400.
- ↑ Amanda M.. Oakley & Karthik Krishnamurthy (2018). "Stevens Johnson Syndrome (Toxic Epidermal Necrolysis)". PMID 29083827. Unknown parameter
|month=ignored (help)