Preferred Name |
Stevens-Johnson syndrome |
|
Synonyms |
Dermatostomatitis, Stevens Johnson type |
|
Definitions |
A limited form of Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum characterized by destruction and detachment of the skin epithelium, involving less than 10% 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. |
|
ID |
http://www.orpha.net/ORDO/Orphanet_36426 |
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alternative_term |
Dermatostomatitis, Stevens Johnson type |
|
definition |
A limited form of Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum characterized by destruction and detachment of the skin epithelium, involving less than 10% 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. |
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definition_citation |
Orphanet |
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expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=en&Expert=36426 |
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has_age_of_onset |
All ages |
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has_inheritance |
Not applicable |
|
hasDbXref |
MedDRA:10042033 ICD-11:EB13.0 UMLS:C0038325 ICD-10:L51.1 OMIM:608579 MeSH:D013262 |
|
label |
Stevens-Johnson syndrome |
|
notation |
ORPHA:36426 Clinical subtype |
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part_of | ||
prefixIRI |
ORDO:Orphanet_36426 |
|
prefLabel |
Stevens-Johnson syndrome |
|
present_in |
Europe AND has_point_prevalence_range : Unknown Europe AND has_annual_incidence_average_value : 0.36 AND has_annual_incidence_range : 1-9 / 1 000 000 |
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treeView | ||
subClassOf |