Stevens-Johnson syndrome (SJS)
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Etiology
- hypersensitivity (most likely)
- possibly immunologic
- drugs & infection most likely triggers
- pharmaceutical agents (~70-80%)[2][10][12]
- antibiotics 32%[13]
- anticonvulsants (19%)[12]
- NSAIDs (12%)[12]
- meloxicam, piroxicam, tenoxicam
- diclofenac, indomethacin, ionazolac, etodolac, aceclofenac, sulindac, ketorolac
- others
- allopurinol (11%)[12], sertraline, pantoprozole, sulfasalazine
- carbonic anhydrase inhibitors used in ophthalmic agents to treat glaucoma
- see pharmaceutical agents causing SCARs
- infections (26%)[2]
Epidemiology
- most common in young adults
- male:female ratio 2:1
Pathology
- acute epidermal necrosis (full thickness)
- involvement of mucous membranes
- potentially fatal due to secondary infection, transcutaneous fluid loss, respiratory complications
* histopathology image[6]
Genetics
- drug-induced SJS (carbamazepine, phenytoin, allopurinol) linked to HLA-B*1502 in Han Chinese (Asians, South Asian Indians)
Clinical manifestations
- prodrome of fever, malaise, arthralgia, headache, respiratory symptoms, vomiting &/or diarrhea 1-14 days before appearance of skin lesions
- skin & mucous membrane manifestations
- diffuse pruritus or burning may occur in the prodromal phase
- early lesions are pink, edematous papules
- these then evolve into dull, red macules with central cyanosis or into vesicles (atypical 'target lesions')
- lesions can coalesce & progress to flaccid bullae with sloughing
- positive Nikolsky sign[14]
- lesions are most prominent on extremities, palms & soles
- erosive lesions may occur on mucous membranes
- oral mucosa, conjunctiva, genitourinary
- mucosa alone may be affected
- hemorrhagic crusting of oral mucosa is characteristic[2]
- systemic manifestations
- time course: 4-6 weeks
Laboratory
- blood cultures
- complete blood count (CBC)
- serum electrolytes, serum urea nitrogen, serum creatinine
- liver function tests
- erythrocyte sedimentation rate may be elevated
- urinalysis
- skin biopsy if diagnosis uncertain
- full-thickness epidermal necrosis[9]
- do not screen for SJS or TEN with HLA-B*1502 & HLA-B*5801[2]
Complications
- epidermal necrosis resulting in infection, sepsis
- mortality is 1-5% (5-13%[2])
- long-term sequella SJS/TEN[11]
- post-inflammatory dsypigmentation: hyperpigmentation, hypopigmentation, or a combination
- hypertrophic or keloidal scars
- nail changes: onycholysis or onychomadesis, onychorrhexis, onychoschizia, koilonychia, erythronychia, oil-drop sign
- nail loss may be permanent (20%)
- hair changes: telogen effluvium is common
- eruptive nevi & atypical nevi
- other cutaneous manifestations: pruritus, hyperhidrosis, photosensitivity, heterotopic ossification, ectopic sebaceous glands
Differential diagnosis
- erythema multiforme
- DRESS syndrome (lymphadenopathy)
- often delayed onset 2-6 weeks
- morbilliform exanthem, facial edema & redness
- generalized exanthematous pustulosis
- erythroderma
- diffuse erythema covering 80% to 90% body surface area
- pruritus, peripheral edema, erosions, scaling, & lymphadenopathy
- toxic epidermal necrolysis (TEN) is a severe form of SJS
- Stevens-Johnson syndrome (SJS) & toxic epidermal necrolysis (TEN) represent a continuum of a single disorder
- in SJS, skin detachment involves < 10% of body area
- in TEN, skin detachment involves > 30% of body area
- in SJS-TEN overlap syndrome, 10-30% of body area involved
Management
- hospitalize; admit to intensive care unit (burn unit)
- identify & eliminate triggering agent
- mild disease (erythema multiforme minor)
- outpatient treatment with topical steroids
- dermatology follow-up
- severe disease (Stevens-Johnson syndrome)
- a short course of high intensity glucocorticoid treatment may be of benefit
- intravenous immune globulin controversial[2]
- cyclosporine has been used[12]
- treat secondary infections
- no role for prophylactic antibiotics[2]
- ophthalmology consult for eye involvement
- prognosis: mortality 10% generally due to infection
More general terms
Additional terms
- erythema multiforme
- pharmaceutical agents causing severe cutaneous adverse reactions (SCARs)
- toxic (bullous) epidermal necrolysis (Lyell syndrome, TEN)
References
- ↑ H. Quinny Cheng, UCSF Fresno lecture, Oct 21, 1998
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 2.8 Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17, 18. American College of Physicians, Philadelphia 2009, 2012, 2015, 2018.
- ↑ Carr DR et al Approach to the acute, generalized, blistering patient. Semin Cutan Med Surg. 2007 Sep;26(3):139-46. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/18070680
- ↑ Cotliar J. Approach to the patient with a suspected drug eruption. Semin Cutan Med Surg. 2007 Sep;26(3):147-54. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/18070681
- ↑ Wetter DA, Camilleri MJ. Clinical, etiologic, and histopathologic features of Stevens- Johnson syndrome during an 8-year period at Mayo Clinic. Mayo Clin Proc. 2010 Feb;85(2):131-8 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20118388
- ↑ 6.0 6.1 6.2 Foster CS, Roy H (images) Medscape: Stevens-Johnson Syndrome http://emedicine.medscape.com/article/1197450-overview
- ↑ 7.0 7.1 DermNet NZ. (images) Stevens Johnson Syndrome & Toxic Epidermal Necrolysis http://www.dermnetnz.org/reactions/sjs-ten.html
- ↑ Fein JD, Hamann KL. Images in clinical medicine. Stevens-Johnson syndrome. N Engl J Med 2005 Apr 22; 352:1696 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/15843672 Free full text <Internet> http://www.nejm.org/doi/full/10.1056/NEJMicm031127
- ↑ 9.0 9.1 NEJM Knowledge+ Question of the Week. Nov 8, 2016 http://knowledgeplus.nejm.org/question-of-week/1452/
- ↑ 10.0 10.1 10.2 Yang MS, Lee JY, Kim J et al Searching for the culprit drugs for Stevens-Johnson syndrome and toxic epidermal necrolysis from a nationwide claim database in Korea. J Allergy Clin Immunol Pract 2020 Feb; 8:690 PMID: https://www.ncbi.nlm.nih.gov/pubmed/31614216 https://www.sciencedirect.com/science/article/abs/pii/S221321981930858X
- ↑ 11.0 11.1 Heymann E Stevens-Johnson Syndrome-Toxic Epidermal Necrolysis: The Aftermath. AAD Reading Room Content MedPage Today. June 22, 2021 https://www.medpagetoday.com/reading-room/aad/general-dermatology/93214
- ↑ 12.0 12.1 12.2 12.3 12.4 12.5 Kridin K, Bruggen MC, Chua SL et al Assessment of Treatment Approaches and Outcomes in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. Insights From a Pan-European Multicenter Study. JAMA Dermatol. Published online August 25, 2021. PMID: https://www.ncbi.nlm.nih.gov/pubmed/34431984 https://jamanetwork.com/journals/jamadermatology/fullarticle/2783034
- ↑ 13.0 13.1 Lee EU, Knox C, Phillips EJ Worldwide Prevalence of Antibiotic-Associated Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Systematic Review and Meta-analysis. JAMA Dermatol. Published online February 15, 2023. PMID: https://www.ncbi.nlm.nih.gov/pubmed/36790777 https://jamanetwork.com/journals/jamadermatology/fullarticle/2801093
- ↑ 14.0 14.1 NEJM Knowledge+ Dermatology
Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis: Part I. Introduction, history, classification, clinical features, systemic manifestations, etiology, and immunopathogenesis. J Am Acad Dermatol. 2013 Aug;69(2):173.e1-13; PMID: https://www.ncbi.nlm.nih.gov/pubmed/23866878 Review.
Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis: Part II. Prognosis, sequelae, diagnosis, differential diagnosis, prevention, and treatment. J Am Acad Dermatol. 2013 Aug;69(2):187.e1-16; PMID: https://www.ncbi.nlm.nih.gov/pubmed/23866879 Review. - ↑ Noe MH, Micheletti RG. Diagnosis and management of Stevens-Johnson syndrome/toxic epidermal necrolysis. Clin Dermatol. 2020;38:607-12. PMID: https://www.ncbi.nlm.nih.gov/pubmed/33341195
- ↑ Charlton OA, Harris V, Phan K, et al. Toxic epidermal necrolysis and Steven-Johnson syndrome: A comprehensive review. Adv Wound Care (New Rochelle). 2020;9:426-439. PMID: https://www.ncbi.nlm.nih.gov/pubmed/32520664