DiseaseID 9893
森特综合征
disease
Ichthyosis hystrix of Curth-Macklin (IHCM) is a rare type of keratinopathic ichthyosis (see this term) that is characterized by the presence of severe hyperkeratotic lesions and palmoplantar keratoder
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Disease: 1Herb: 9Symptom: 12Target: 24Links: 45
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Record Fields
Scalar fields from the final disease record.
- Disease Id
- 9893
- Core Entity Id
- 67027
- Source Entity Count
- 1
- Preferred Name
- Senter Syndrome
- Name Cn
- 森特综合征
- Name Pinyin
- Sen Te Zong He Zheng
- Name En
- Senter Syndrome
- Name Latin
- Bilingual Status
- complete
- Disease Type
- disease
- Umls Disease Type
- Congenital Abnormality
- Disgenet Type
- disease
- Mesh Class
- Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nervous System Diseases; Eye Diseases; Skin and Connective Tissue Diseases; Pathological Conditions, Signs and Symptoms; Otorhinolaryngologic DiseasesCongenital, Hereditary, and Neonatal Diseases and Abnormalities; Nervous System Diseases; Skin and Connective Tissue Diseases; Pathological Conditions, Signs and Symptoms; Otorhinolaryngologic DiseasesEye DiseasesSkin and Connective Tissue Diseases; Congenital, Hereditary, and Neonatal Diseases and Abnormalities
- Do Class
- disease of anatomical entitydisease of anatomical entity; genetic diseasegenetic disease
- Hpo Class
- Mesh Class Name
- Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Skin and Connective Tissue DiseasesEye DiseasesPathological Conditions, Signs and Symptoms; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Eye Diseases; Skin and Connective Tissue Diseases; Nervous System Diseases; Otorhinolaryngologic DiseasesPathological Conditions, Signs and Symptoms; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Skin and Connective Tissue Diseases; Nervous System Diseases; Otorhinolaryngologic Diseases
- Hpo Class Name
- Do Class Name
- disease of anatomical entitygenetic diseasegenetic disease; disease of anatomical entity
- Disease Definition
- Ichthyosis hystrix of Curth-Macklin (IHCM) is a rare type of keratinopathic ichthyosis (see this term) that is characterized by the presence of severe hyperkeratotic lesions and palmoplantar keratoder
- Version
- v1
- Suppressed
- No
Names
Preferred names, aliases, and source labels retained in the final schema.
Name
Senter Syndrome
Role
preferred
Name
Ichthyosis, Hystrix-Like, With Deafness
Role
preferred
Name
Keratitis-Ichthyosis-Deafness Syndrome
Role
preferred
Name
Ichthyosis Hystrix Of Curth-Macklin
Role
preferred
Name
Ichthyosis With Hypotrichosis, Autosomal Recessive
Role
preferred
Name
Ichthyosis-Hypotrichosis Syndrome
Role
preferred
Name
Kid Syndrome
Role
preferred
Name
Netherton Syndrome
Role
preferred
Name
Other Congenital Ichthyosis
Role
preferred
Name
PEELING SKIN SYNDROME
Role
preferred
Name
Peeling Skin Syndrome 2
Role
preferred
Name
Autosomal Dominant Keratitis-Ichthyosis-Deafness Syndrome
Role
alias
Name
Autosomal Recessive Congenital Ichthyosis 11
Role
alias
Name
HID SYNDROME
Role
alias
Name
Hystrix-Like Ichthyosis With Deafness
Role
alias
Name
Ichthyosis, Congenital, Autosomal Recessive 11
Role
alias
Name
Keratitis, Ichthyosis, And Deafness (Kid) Syndrome
Role
alias
Name
Keratitis-Ichthyosis-Deafness Syndrome, Autosomal Dominant
Role
alias
Name
Peeling Skin Syndrome 1
Role
alias
Cross References
Trusted external identifiers retained for this final record.
Herb
HBDIS005820HBDIS009437HBDIS009718HBDIS015356HBDIS016570HBDIS018189HBDIS021940
Omim
148210242150270300602400602540
Umls
C0265962C0432307C1835851C1840296C1853354C1865234C3665333
Icd10
Q80.8
Med Dra
1004878610062909
Sym Map
SMDE00235SMDE00636SMDE00724SMDE01375SMDE01389SMDE03390SMDE10175SMDE12010
Do Class
DOID:630DOID:7
Dis Ge Net
C0265336C0432307C0478084C1835851C1849193C1865234C3665333
Orphanet
2635344776347950391132
Umls Sty
T019T047
Me Sh Class
C09C10C11C16C17C23
Etcm Disease
Ichthyosis, Hystrix-Like, with Deafness
Tcmbank Disease
1120817712183502134426917274664955
Itcmdb Generated
ITX-DISEASE-F56ACBC33512
Attributes
Merged source attributes and domain-specific metadata.
Version
v1v1,v2v2
Suppress
0
Page Title
Disease Ichthyosis, Hystrix-Like, with Deafness Details page
Do Class Name
disease of anatomical entitygenetic diseasegenetic disease; disease of anatomical entity
Disease Type
disease
Do Disease Class
disease of anatomical entitydisease of anatomical entity; genetic diseasegenetic disease
Umls Disease Type
Congenital AbnormalityDisease or SyndromeDisease or Syndrome; Congenital Abnormality
Basic Information
Disease Name
Ichthyosis, Hystrix-Like, with Deafness
Global Category
Cancer diseases;Genetic diseases
Anatomical Category
Ear diseases;Skin diseases
Disease Definition
Ichthyosis hystrix of Curth-Macklin (IHCM) is a rare type of keratinopathic ichthyosis (see this term) that is characterized by the presence of severe hyperkeratotic lesions and palmoplantar keratoderIchthyosis-hypotrichosis syndrome is characterised by congenital ichthyosis and hypotrichosis. It has been described in three members of a consanguineous Arab Israeli family. The syndrome is transmittKeratitis (and hystrix-like) ichthyosis deafness (KID/HID) syndrome is a rare congenital ectodermal disorder characterized by vascularizing keratitis, hyperkeratotic skin lesions and hearing loss.Netherton syndrome (NS) is a skin disorder characterized by congenital ichthyosiform erythroderma (CIE), a distinctive hair shaft defect (trichorrhexis invaginata; TI) and atopic manifestations.
Me Sh Disease Class
Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nervous System Diseases; Eye Diseases; Skin and Connective Tissue Diseases; Pathological Conditions, Signs and Symptoms; Otorhinolaryngologic DiseasesCongenital, Hereditary, and Neonatal Diseases and Abnormalities; Nervous System Diseases; Skin and Connective Tissue Diseases; Pathological Conditions, Signs and Symptoms; Otorhinolaryngologic DiseasesEye DiseasesSkin and Connective Tissue Diseases; Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Dis Ge Net Disease Type
disease
Disease Class Name Me Sh
Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Skin and Connective Tissue DiseasesEye DiseasesPathological Conditions, Signs and Symptoms; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Eye Diseases; Skin and Connective Tissue Diseases; Nervous System Diseases; Otorhinolaryngologic DiseasesPathological Conditions, Signs and Symptoms; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Skin and Connective Tissue Diseases; Nervous System Diseases; Otorhinolaryngologic Diseases
Umls Semantic Type Name
Congenital AbnormalityDisease or SyndromeDisease or Syndrome; Congenital Abnormality