Code System Concept

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Code System Concept

Code System Concept Code	763775000
Code System Concept Name	Keratosis linearis, ichthyosis congenita, sclerosing keratoderma syndrome (disorder)
Code System Preferred Concept Name	Keratosis linearis, ichthyosis congenita, sclerosing keratoderma syndrome (disorder)
Concept Status	Published
Concept Status Date	09/01/2024
Code System Name	SNOMED-CT

Concept Relationships
Concept Details

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Superseded By Concept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	07/31/2018
SDO Date Created	07/31/2018
SDO Date Revised	07/31/2018
Description	An inherited epidermal disorder with characteristics of palmoplantar keratoderma, linear hyperkeratotic papules on the flexural side of large joints (cord-like distribution around wrists, in antecubital and popliteal folds), hyperkeratotic plaques (on neck, axillae, elbows, wrists, and knees), mild ichthyosiform scaling, and sclerotic constrictions around fingers that present flexural deformities. The disease is caused by homozygous mutation in the POMP gene.
Sequence

Extended Properties

No extended properties.

Concept Alternate Designations

SDO Designation ID	Concept Designation Description	Status	Designation Type	PHIN Preferred Term Flag	Code Flag
3643799015	KLICK syndrome	Active	Synonym	false	false
3643801017	Keratosis linearis, ichthyosis congenita, sclerosing keratoderma syndrome	Active	Synonym	false	false

Associated Value Sets

Value Set Name	Version(s)
Clinical Finding (NNDSS)	2 , 1
Disease	10 , 9 , 8
Problem Value Set	3

Parent Concepts Parent Concepts Parent/Child (Relationship Type) Autosomal recessive ichthyosis (disorder) {402772005 , SNOMED-CT } Erythrokeratodermia variabilis (disorder) {70041004 , SNOMED-CT } Hereditary diffuse palmoplantar keratoderma (disorder) {400123002 , SNOMED-CT } Mutilating keratoderma (disorder) {24559001 , SNOMED-CT } Rough skin of hands (finding) {829993001 , 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:
- Centers for Disease Control and Prevention,