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Potentially life-threatening emergency
Stevens-Johnson syndrome - External and Internal Eye
See also in: Overview,Oral Mucosal Lesion
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Potentially life-threatening emergency

Stevens-Johnson syndrome - External and Internal Eye

See also in: Overview,Oral Mucosal Lesion
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Contributors: Brandon D. Ayres MD, Christopher Rapuano MD, Harvey A. Brown MD, Sunir J. Garg MD, Lauren Patty Daskivich MD, MSHS
Other Resources UpToDate PubMed

Synopsis

Stevens-Johnson syndrome (previously named erythema multiforme [EM] major) is an acute, severe hypersensitivity reaction, usually triggered by drugs or infection. Implicated drugs include sulfonamides, phenytoin and other anticonvulsants, allopurinol, penicillins, and non-steroidal anti-inflammatory drugs. Susceptibility to phenytoin-induced Stevens-Johnson syndrome and toxic epidermal necrolysis was associated with genetic factors in a study of patients in Taiwan, Japan, and Malaysia. More severe cases may be expected with Stevens-Johnson related to sulfa drugs, gold, and phenylbutazone. The best documented association of Stevens-Johnson syndrome with an infection is with mycoplasma pneumonia. Herpes simplex virus (HSV) infection may be associated. Other viral associations include influenza, orf, Coxsackie, ECHO, and Epstein-Barr virus. Yersinia, tuberculosis, histoplasmosis, coccidioidomycosis, and X-ray therapy of certain tumors are well-documented associations. Women appear to be twice as commonly affected as men.

Stevens-Johnson syndrome is distinguished from EM minor by the extent of the cutaneous and mucous membrane involvement and the usual presence of target or "bull's eye"-configured plaques in EM minor. While Stevens-Johnson syndrome is typically triggered by medications, EM minor is more frequently triggered by viral infections such as herpes infections. In Stevens-Johnson syndrome, the plaques may be of varying morphologies without target shapes. Stevens-Johnson syndrome has involvement of the mucous membranes while EM minor does not.

Fever, malaise, rhinitis, pharyngitis, and cough may precede the rash. Myalgias, vomiting, diarrhea, and arthralgias may also occur. Cases with pneumonia or characteristics of toxic epidermal necrolysis may result in death. Recurrences of Stevens-Johnson syndrome are uncommon.

Ocular involvement in Stevens-Johnson syndrome occurs in over 50% of patients. Conjunctivitis is the most common eye finding.

Swelling of the hands, feet, and lips may be seen. Esophageal strictures, anal strictures, vaginal stenosis, and urethral meatal stenosis may be seen. GI bleeding, hepatitis, urinary retention, nephritis, anuria from dehydration and genitourinary injury, myocarditis, pneumothorax, obtundation, and seizures are rare complications. Other involvement includes GI lesions with diarrhea, cystitis, splenic inflammation, arthritis, pneumonitis, otitis media, paronychia, and nail shedding.

Codes

ICD10CM:
L51.1 – Stevens-Johnson syndrome

SNOMEDCT:
73442001 – Stevens-Johnson syndrome

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Erythema multiforme – Histologic features may not differentiate EM from SJS/TEN. Clinically, however, EM has characteristic target lesions (3 concentric colors that are round and well demarcated) that occur on the extremities more often than the trunk. Precipitating factors are usually infectious (HSV, mycoplasma, etc) and not medications. Lesions may be papular. Note that EM is not considered within the same disease spectrum as SJS/TEN and confers no risk in progressing to TEN. Nikolsky sign negative.
  • Staphylococcal scalded skin syndrome – Usually occurs in newborns, infants, and young children; mucous membranes and palms/soles are spared. The exfoliated skin is significantly more superficial (subcorneal vs epidermal-dermal). Also look for purulent discharge from the nose, and histologically very different from SJS/TEN. Nikolsky sign can be positive.
  • Acute generalized exanthematous pustulosis (AGEP) – Look for neutrophilia, eosinophilia, almost confluent erythema with overlying non-follicular pustules. Nikolsky sign can be positive. Histology will clearly differentiate AGEP from SJS/TEN.
  • Linear IgA dermatosis – Look for tense blisters; histology will help differentiate Linear IgA from SJS/TEN. Direct immunofluorescence will demonstrate linear IgA deposition. DIF is negative in SJS/TEN.
  • Toxic shock syndrome and toxic-shock-like syndrome – Look for sudden onset of exanthematous eruption. Histology will help differentiate TSS and SJS/TEN.
  • Drug hypersensitivity syndrome (DRESS) – Look for facial edema (hallmark of DRESS), eosinophilia, hepatitis, and other viscera.
  • Pemphigus vulgaris
  • Paraneoplastic pemphigus – Significant involvement of the vermillion border.

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Updated: 11/07/2014
Copyright © 2018 VisualDx®. All rights reserved.
Potentially life-threatening emergency
Stevens-Johnson syndrome - External and Internal Eye
See also in: Overview,Oral Mucosal Lesion
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Stevens-Johnson syndrome : Bullae, Eyelids, Lip mucosa hemorrhagic crust, Lips, Patient appears ill, Widespread, Conjunctival injection, Vesicles
Clinical image of Stevens-Johnson syndrome
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