Stevens-Johnson syndrome

Stevens-Johnson syndrome (SJS) is a rare but serious disorder that affects the skin and mucous membranes.

Flu-like symptoms (fever, sore throat, fatigue) appear first.

Followed by a painful red or purplish rash that spreads and blisters.

Skin starts to peel, and large areas may slough off.

Mucous membranes (eyes, mouth, genital area) are often severely affected.

It is usually triggered by a reaction to medication or, less commonly, by an infection. SJS is considered part of a spectrum with toxic epidermal necrolysis (TEN)—SJS being the milder form and TEN the more severe.

Medications: Most commonly sulfa antibiotics, anticonvulsants (like lamotrigine), allopurinol, NSAIDs, etc.

Infections: Especially Mycoplasma pneumoniae in children and young adults.

In some cases, the cause remains unknown.

Adverse Reactions to COVID-19 Vaccination ¹⁾.

Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rare, severe skin reactions, usually triggered by medications or infections. They are considered different levels of the same disease spectrum.

• Medications (most common):
  • Sulfonamides
  • Anticonvulsants (e.g., lamotrigine, carbamazepine)
  • Allopurinol
  • NSAIDs
• Infections:
  • *Mycoplasma pneumoniae* (especially in children)
• Unknown cause in some cases

• Fever, malaise (flu-like prodrome)
• Painful red/purplish rash
• Blistering and peeling of skin
• Involvement of mucous membranes:
  • Eyes (conjunctivitis)
  • Mouth (ulcerations)
  • Genitals

• Immediate withdrawal of suspected drug
• Hospital admission (ICU or burn unit)
• Skin care, fluid and electrolyte support
• Pain management
• Prevention of secondary infections
• Sometimes: corticosteroids, IVIG, cyclosporine

<folded|Click to expand: Possible long-term effects>

• Chronic skin problems
• Ocular damage (e.g., dry eye, blindness)
• Oral/genital mucosal scarring
• Psychological trauma

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• Roujeau JC, Stern RS. Severe adverse cutaneous reactions to drugs. _N Engl J Med._ 1994.
• Harr T, French LE. Toxic epidermal necrolysis and Stevens-Johnson syndrome. _Orphanet J Rare Dis._ 2010.
• [https://www.uptodate.com/contents/stevens-johnson-syndrome-toxic-epidermal-necrolysis | UpToDate Entry]

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Immediate discontinuation of the suspected causative drug.

Supportive care, often in an intensive care unit or burn unit.

Wound care, fluid and electrolyte management, pain control, and prevention of secondary infections.

Sometimes immunosuppressive therapies (e.g., corticosteroids, IVIG, or cyclosporine) are used, although evidence is mixed.

Mortality: ~5–10% for SJS, up to 30–50% for TEN.

Long-term complications can include chronic skin issues, ocular damage (e.g., blindness), and genital or oral scarring.

• Caring for Coma after Severe Brain Injury: Clinical Practices and Challenges to Improve Outcomes: An Initiative by the Curing Coma Campaign
• Toxic epidermal necrolysis caused by phenobarbital: a case report and literature review
• Genetic variants for head size share genes and pathways with cancer
• Research considerations for prospective studies of patients with coma and disorders of consciousness
• Global Burden of Cardiovascular Diseases and Risks, 1990-2022
• Carbamazepine-modified HLA-A*24:02-bound peptidome: Implication of CORO1A in skin rash
• Patients With Suspected Severe Adverse Reactions to COVID-19 Vaccination Admitted to Intensive Care Unit: A Case Report
• Phenytoin induced Stevens-Johnson Syndrome-toxic Epidermal Necrolysis Overlap Exacerbated by Cephalexin in a 65-Year-old Neurosurgical Patient: A Rare Case Report

A 65-year-old female who had come to the hospital with complaints of Sloughing of the skin and redness all over the body with raised body temperature. She was on therapeutic Phenytoin to prevent the post-surgical complications of Communicating Hydrocephalus. After a detailed examination, it was found that the patient had been misemployed with an overdose of Phenytoin. The patient was found with nikolsky sign and diagnosed with Stevens-Johnson syndrome and Toxic Epidermal Necrosis overlap. This case report emphasizes phenytoin-induced Stevens-Johnson syndrome and Toxic Epidermal Necrosis syndrome exacerbated by cephalexin.

Practice implications: By witnessing this phenomenon, we could figure out the association between cephalexin and Stevens-Johnson syndrome- Toxic Epidermal Necrosis syndrome overlap. The immediate dismissal of the offending agent and commencement of supportive care was found to be effective ²⁾.