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Toxic epidermal necrolysis

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Disease definition

An extended form of Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum characterized by destruction and detachment of the skin epithelium, involving 30% or more of the body surface area, and mucous membranes. Onset usually occurs 4-28 days after administration of the causal medication and is most frequently associated with anticonvulsants, antibacterial sulfonamides, allopurinol, nevirapine, and oxicams (non-steroidal anti-inflammatory drugs), but many other medications have also been implicated. The disease is not induced by medication in 15% of cases. Histology is characterized by an epidermal necrolysis. Multiple disabling long-term sequelae (especially cutaneous, ocular and psychological) are frequent.

ORPHA:537

Classification level: Subtype of disorder

Synonym(s):
  • Lyell syndrome

Prevalence: Unknown

Inheritance: Not applicable

Age of onset: All ages

ICD-10: L51.2

ICD-11: EB13.1

UMLS: C0014518

MedDRA: 10044223

A summary on this disease is available in Français, Español, Deutsch, Italiano, Português, Nederlands, Polski
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

Guidelines
Emergency guidelines
Français (2019.pdf) - Orphanet Urgences
English (2019.pdf) - ERN-Skin Logo ERN
Italiano (2012.pdf) - Orphanet Urgences
Clinical practice guidelines
English (2023) - Orphanet J Rare Dis Logo ERN
English (2019) - Br J Dermatol Logo ERN
English (2020) - Orphanet J Rare Dis Logo ERN
English (2020) - J Am Acad Dermatol Logo ERN
English (2016) - Br J Dermatol Logo ERN
English (2021) - Br J Dermatol Logo ERN
Disease review articles
Review article
Français (2007.pdf) - Rev Praticien
English (2025) - J Invest Dermatol
English (2010) - Orphanet J Rare Dis
The documents contained in this website are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.