Application Version: 4.0.6.10
Content Version: 2024.07.12
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Code System Concept
Code System Concept Code | 784339002 |
Code System Concept Name | Deficiency of interleukin 36 receptor antagonist (disorder) |
Code System Preferred Concept Name | Deficiency of interleukin 36 receptor antagonist (disorder) |
Concept Status | Published |
Concept Status Date | 09/01/2024 |
Code System Name | SNOMED-CT |
Concept Details
Code System Code | PH_SNOMED-CT |
Code System OID | 2.16.840.1.113883.6.96 |
HL7 Table 0396 Code | SCT |
Superseded By Concept | |
SDO Concept Status | Active |
SDO Concept Status Date | 07/31/2019 |
SDO Date Created | 07/31/2019 |
SDO Date Revised | 07/31/2019 |
Description | A rare genetic autoinflammatory syndrome with immune deficiency disease characterized by recurrent and severe flares of generalized pustular psoriasis associated with high fever, asthenia and systemic inflammation due to IL36R antagonist deficiency. Psoriatic nail changes (for example pitting and onychomadesis) and ichthyosis may occasionally be associated. |
Sequence |
Extended Properties
No extended properties.
Concept Alternate Designations
SDO Designation ID | Concept Designation Description | Status | Designation Type | PHIN Preferred Term Flag | Code Flag |
---|---|---|---|---|---|
3763674018 | DITRA - deficiency of interleukin 36 receptor antagonist | Active | Synonym | false | false |
3763675017 | Deficiency of IL-36R antagonist | Active | Synonym | false | false |
3763676016 | Deficiency of interleukin 36 receptor antagonist | Active | Synonym | false | false |
3763678015 | Deficiency of IL-36Ra (interleukin 36 receptor antagonist) | Active | Synonym | false | false |
Parent Concepts
Parent Concepts
Parent/Child (Relationship Type)
Acute blistering eruption of skin (disorder)
{721542002
, SNOMED-CT
}
Autoinflammatory disease (disorder)
{42111000175103
, SNOMED-CT
}
Autosomal recessive hereditary disorder (disorder)
{85995004
, SNOMED-CT
}
Chronic disease of immune structure (disorder)
{413835007
, SNOMED-CT
}
Generalized pustular psoriasis (disorder)
{238612002
, SNOMED-CT
}
Hereditary disorder of immune system (disorder)
{363138005
, SNOMED-CT
}
Hereditary disorder of the integument (disorder)
{363185004
, SNOMED-CT
}
Inflammatory disorder (disorder)
{128139000
, SNOMED-CT
}
Primary immune deficiency disorder (disorder)
{58606001
, SNOMED-CT
}
Vesicular eruption (disorder)
{79893008
, SNOMED-CT
}
|
Other Relationships
No other relationships present.
|
Child Concepts
No child concepts present.
|
- Page last reviewed:June 20, 2024
- Page last updated:June 20, 2024
- Content source: