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Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome
Disease definition
An intermediate form of Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum characterized by destruction and detachment of the skin epithelium, involving between 10 to 29% 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:506784
Classification level: Subtype of disorder- Synonym(s):
- SJS/TEN overlap syndrome
- Stevens-Johnson/TEN overlap syndrome
- Stevens-Johnson/toxic epidermal necrolysis overlap syndrome
- Prevalence: -
- Inheritance: -
- Age of onset: -
- ICD-10: L51.2
- ICD-11: EB13.2
- OMIM: -
- UMLS: -
- MeSH: -
- GARD: -
- MedDRA: -
A summary on this disease is available in Español (2022) Français (2022) Nederlands (2022) Polski ()
Detailed information
Guidelines
- Emergency guidelines
- Français (2019, pdf) - Orphanet Urgences
- English (2019, pdf) - ERN-Skin
- Clinical practice guidelines
- English (2016) - Br J Dermatol
- English (2019) - Br J Dermatol
- English (2020) - J Am Acad Dermatol
- English (2020) - Orphanet J Rare Dis
- Français (2021) - PNDS
- English (2021) - Br J Dermatol
- English (2023) - Orphanet J Rare Dis
produced/endorsed by ERN(s) produced/endorsed by FSMR(s)Additional information