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Stevens-Johnson Syndrome

Stevens-Johnson Syndrome is a topic covered in the 5-Minute Clinical Consult.

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Basics

Description

  • A generalized hypersensitivity reaction, usually to a drug, in which skin and mucous membrane lesions are an early manifestation
  • A severe form of erythema multiforme in which >1 mucosal surface is involved; many now consider it to be a different disease with a more difficult course and a more ominous prognosis.
  • Stevens-Johnson syndrome (SJS) exists on a continuum with toxic epidermal necrolysis (TEN).
  • SJS presents with characteristic targetoid cutaneous lesions when <10% of the body surface area (BSA) is involved.
  • Targetoid cutaneous lesion involvement of 10–30% of BSA is considered an overlap between SJS and TEN. Involvement of >30% of BSA is TEN, which has a high morbidity and up to 70% mortality.
  • System(s) affected: cardiovascular, hematologic, lymphatic/immunologic, nervous, renal/urologic, skin/exocrine
  • Synonym(s): ectodermosis erosiva pluriorificialis; febrile mucocutaneous syndrome; herpes iris; erythema polymorphe; TEN

ALERT
Patients with discrete skin lesions and >10% epidermal detachment are at risk of rapid progression to TEN.

Geriatric Considerations
TEN has a greater mortality in older patients.

Pediatric Considerations
Rare in children <3 years of age, more common from age 3 years to young adult

Pregnancy Considerations
Pregnancy is a possible predisposing condition.

Epidemiology

Incidence
  • Incidence/prevalence of SJS in the United States is difficult to estimate due to the lack of a universally accepted case definition.
  • 1.1 to 7.1 and 0.4 to 1.2 cases/1 million person-years for SJS and TEN, respectively

Prevalence
  • Predominant sex: male > female (2:1)
  • Sex (females): 33–62% for SJS and 61–64% for TEN
  • Age (average range): 25 to 47 years for SJS and 46 to 63 years for TEN; <10% of cases are children.

Etiology and Pathophysiology

  • 50% of cases are idiopathic; cross-reactivity between drug classes is suspected (1).
  • Associated with abnormal metabolism and clearance of drugs and metabolites (2)
  • Slow intrinsic acetylation rates
  • 75% of cases involving SJS/TEN overlap with recent medication:
    • 50% of cases occur within 4 weeks and 90% occur within 8 weeks of an offending agent (1).
  • Algorithm-based approach helps to assess probability of drug causality in SJS, TEN, or overlap (3)[B],(4).
  • Frequently associated with upper respiratory tract infection and recent use of antibiotics
  • Sulfonamides are the drugs most strongly associated with SJS and TEN. Others include:
    • Cephalosporins
    • Quinolones
    • Aminopenicillins
    • Tetracyclines
    • Macrolides
    • Imidazole antifungals
    • Antiretrovirals (e.g., protease inhibitors, efavirenz, abacavir, amprenavir, telaprevir, fosamprenavir, atazanavir, darunavir, etravirine)
    • Anticonvulsants, especially carbamazepine
    • NSAIDs, especially oxicam
    • Allopurinol, especially in patients of European/Israeli heritage
    • Acetaminophen
    • Vaccines: DPT, BCG, oral polio
    • Mycoplasma pneumoniae infection
  • Erythematous papular lesions and keratinocyte necrosis are a consequence of cell-mediated immunity.
  • Occurs 1 to 2 weeks after initial exposure to offending drugs and within 48 hours on rechallenge
  • Accumulation and binding of reactive drug metabolites to mucocutaneous epithelial cells as haptens (3)
  • Drug haptens signal drug-specific CD8+ T lymphocyte and macrophages, which infiltrate and express interleukin 2 (IL-2), tumor necrosis factor-α (TNF-α), and interferon-γ, leading to keratinocyte activation.
  • Cytokines enhance keratinocyte expression of soluble Fas ligand and Fas receptors, leading to apoptosis.

Genetics
Associations with HLA-A*3101 in Northern Europeans; HLA-B*1501, HLA-B*1502, HLA-B*1511, HLA-B*5801 in patients of Asian ancestry, HLA-Bw44, HLA-B12, and HLA-DQB1*0601

Risk Factors

  • Previous history of SJS
  • Immunocompromised status, including chronic viral infections with Epstein-Barr virus and HIV (5)
  • Patients with HIV infection may be predisposed to developing SJS in response to their medications:
    • HLA allele subtypes A, B, and D
    • Diseases that cause immune compromise (e.g., deficiencies, malignancy)
    • Possibly radiation therapy/ultraviolet light

General Prevention

Secondary prevention possible by avoiding exposure to offending medications/chemical agents

Commonly Associated Conditions

  • SJS progressing to TEN is ominous prognostically.
  • M. pneumoniae may be an infectious precursor.

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Citation

Stephens, Mark B., et al., editors. "Stevens-Johnson Syndrome." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2019. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816574/all/Stevens_Johnson_Syndrome.
Stevens-Johnson Syndrome. In: Stephens MB, Golding J, Baldor RA, et al, eds. 5-Minute Clinical Consult. 27th ed. Wolters Kluwer; 2019. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816574/all/Stevens_Johnson_Syndrome. Accessed April 24, 2019.
Stevens-Johnson Syndrome. (2019). In Stephens, M. B., Golding, J., Baldor, R. A., & Domino, F. J. (Eds.), 5-Minute Clinical Consult. Available from https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816574/all/Stevens_Johnson_Syndrome
Stevens-Johnson Syndrome [Internet]. In: Stephens MB, Golding J, Baldor RA, Domino FJ, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2019. [cited 2019 April 24]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816574/all/Stevens_Johnson_Syndrome.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Stevens-Johnson Syndrome ID - 816574 ED - Stephens,Mark B, ED - Golding,Jeremy, ED - Baldor,Robert A, ED - Domino,Frank J, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816574/all/Stevens_Johnson_Syndrome PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -