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Potentially life-threatening emergency
Stevens-Johnson syndrome in Infant/Neonate
See also in: External and Internal Eye,Oral Mucosal Lesion
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Other Resources UpToDate PubMed
Potentially life-threatening emergency

Stevens-Johnson syndrome in Infant/Neonate

See also in: External and Internal Eye,Oral Mucosal Lesion
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Contributors: Craig N. Burkhart MD, Dean Morrell MD, Lowell A. Goldsmith MD, MPH
Other Resources UpToDate PubMed

Synopsis

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are mucocutaneous diseases characterized by the acute onset of mucosal erosions with skin pain and detachment. Unlike children and adults, in whom drugs are the major etiology, all reported cases in infants younger than 6 months of age have been due to either bacterial sepsis combined with multi-antibiotic regimens or acute graft-versus-host disease (GVHD). Acute GVHD in infants is usually secondary to engraftment of maternally transmitted or transfusion-derived T lymphocytes in infants with severe combined immunodeficiency (SCID). Drugs are a more important etiology in infants older than 6 months of age. The prognosis of SJS/TEN in infants has not been studied, but thus far has been fatal in infants younger than 6 months of age.

Note: In infants and neonates, SJS/TEN is exceedingly rare. Fewer than 10 case reports exist. Cases were often associated with gram-negative bacteremias and lack confirmatory histopathology. Consideration of more common diagnoses should be carefully evaluated. See Differential Diagnosis & Pitfalls section.

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

Note: In infants and neonates, SJS/TEN is exceedingly rare. Fewer than 10 case reports exist. Cases were often associated with gram-negative bacteremias and lack confirmatory histopathology. Consideration of more common diagnoses, including EM, MIRM, staphylococcal scalded skin, epidermolysis bullosa, and purpura fulminans, should be carefully evaluated.

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: 05/15/2019
Copyright © 2019 VisualDx®. All rights reserved.
Potentially life-threatening emergency
Stevens-Johnson syndrome in Infant/Neonate
See also in: External and Internal Eye,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
Erosions and hemorrhagic crusting on and around the lips, and some erosions on the tongue, in a case of SJS induced by an anticonvulsant.
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