Stevens-Johnson-syndroom op spectrum van toxische epidermale necrolyse (aandoening)
Stevens-Johnson-syndroom op spectrum van toxische epidermale necrolyse
Stevens-Johnson syndrome, toxic epidermal necrolysis spectrum
Toxic epidermolysis
An acute and severe skin disease with clinical and histological features of destruction and detachment of the skin epithelium and mucous membranes. Onset may occur at any age, but the risk increases after 40 years. Three subforms have been described according to the percentage of the body surface area affected: Stevens-Johnson syndrome (less than 10%), Lyell syndrome (greater than 30%) and an intermediate form (10-29%). The initial manifestations are nonspecific: a seemingly banal rash, fever, and a burning sensation involving the eyes, mouth and genitalia. The rash rapidly progresses to become vesicular and bullous on the face and body. High fever is a constant feature. Two thirds of cases are triggered by a drug allergy. In rare cases, the disease is associated with infections or bone marrow transplantation. The remaining 25-30% of cases are classed as idiopathic. The prognosis for patients with extensive forms is poor.
Associated morphologynecrose
Finding sitestructuur van huid
Associated morphologyerytheem
Finding sitestructuur van huid
Associated morphologydiastase
Finding sitestructuur van huid en/of slijmvlies
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TermToxische epidermale necrolyse [Lyell]
SNOMED CT to Orphanet simple map95455
SNOMED CT to ICD-10 extended map
AdviceALWAYS L51.2
CorrelationSNOMED CT source code to target map code correlation not specified