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Toxic Epidermal Necrolysis

Severe drug-induced mucocutaneous reaction with widespread epidermal detachment

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dermatoloji department. Book Appointment →

What is Toxic Epidermal Necrolysis?

Toxic epidermal necrolysis (TEN) is the most severe form of severe cutaneous adverse reactions, characterized by widespread keratinocyte apoptosis leading to epidermal detachment of more than 30% of total body surface area. Stevens-Johnson syndrome (SJS), SJS-TEN overlap and TEN form a continuum based on the extent of skin detachment.

Pathogenesis involves a CD8+ T-cell-mediated cytotoxic reaction with granulysin, perforin, granzyme B and FasL release. Common culprit drugs include allopurinol, anticonvulsants (carbamazepine, phenytoin, lamotrigine), sulfonamides, NSAIDs and nevirapine. Mortality reaches 30-50% in TEN despite optimal care.

Symptoms

Prodromal fever and malaise 1-3 days before rash
Painful erythematous macules progressing to bullae
Nikolsky sign positive (skin slides off with pressure)
Mucosal involvement (oral, ocular, genital)
Severe odynophagia and inability to eat
Conjunctivitis and photophobia
Hemorrhagic crusting of lips
Sheets of epidermal detachment exposing dermis

Risk Factors

HLA-B*15:02 (carbamazepine, Asian population)
HLA-B*58:01 (allopurinol)
HIV infection (1000-fold higher risk)
Slow acetylator phenotype
Female sex
Lupus erythematosus and autoimmune disorders
Recent radiation therapy
Polypharmacy especially anticonvulsants and antibiotics

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Painful skin rash within 1-4 weeks of starting new drug
  • Blistering or peeling skin (emergency)
  • Mouth, eye or genital sores with rash
  • Fever with sore throat and rash
  • Skin pain disproportionate to appearance
  • Targetoid lesions evolving to bullae
  • Any suspected drug allergy with mucosal involvement

Treatment Methods

01
Immediate withdrawal of all suspect medications
02
Burn unit or ICU admission for SCORTEN-graded patients
03
Aggressive fluid and electrolyte replacement (Parkland-style)
04
Sterile wound care with non-adherent dressings
05
Empirical broad-spectrum antibiotics only with documented infection
06
Cyclosporine 3-5 mg/kg/day for 7-10 days as first-line immunomodulator
07
Etanercept or other anti-TNF agents in selected cases
08
Ophthalmology consultation for ocular involvement and amniotic membrane
09
Aggressive nutritional support and pain control

Which Department to Visit?

You can visit our Dermatoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Dermatoloji Department

Let us help you

You can make an appointment with our specialists or contact us for your concerns.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.