DiseaseID 841

家族性苔藓样淀粉样变性

disease

SNOMEDCT_US_2016_09_01:A rare chronic form of cutaneous amyloidosis, a skin disease with characteristics of the accumulation of amyloid deposits in the dermis. Clinical manifestations include the development of pruritic,

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Disease: 1Formula: 13Herb: 12Symptom: 1Target: 20Links: 50
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Record Fields

Scalar fields from the final disease record.

Disease Id
841
Core Entity Id
944
Source Entity Count
1
Preferred Name
Familial Lichen Amyloidosis
Name Cn
家族性苔藓样淀粉样变性
Name Pinyin
Jia Zu Xing Tai Xian Yang Dian Fen Yang Bian Xing
Name En
Familial lichen amyloidosis
Name Latin
Bilingual Status
complete
Disease Type
disease
Umls Disease Type
Disease or Syndrome
Disgenet Type
disease
Mesh Class
Skin and Connective Tissue Diseases; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases
Do Class
disease of anatomical entity; disease of metabolism
Hpo Class
Abnormality of metabolism/homeostasis
Mesh Class Name
Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Skin and Connective Tissue Diseases
Hpo Class Name
Abnormality of metabolism/homeostasis
Do Class Name
disease of metabolism; disease of anatomical entity
Disease Definition
SNOMEDCT_US_2016_09_01:A rare chronic form of cutaneous amyloidosis, a skin disease with characteristics of the accumulation of amyloid deposits in the dermis. Clinical manifestations include the development of pruritic, often pigmented hyperkeratotic papules on trunk and extremities, especially on the shins. Histological findings include the deposition of amyloid or amyloid-like proteins in the papillary dermis.|HPO2016_07_04:The presence of amyloid deposition in the superficial dermis. [HPO:probinson, pmid:19690585]
Version
v1,v2
Suppressed
No

Names

Preferred names, aliases, and source labels retained in the final schema.

Name
Familial Lichen Amyloidosis
Role
preferred
Name
Amyloidosis, Primary Localized Cutaneous, 1
Role
preferred
Name
Amyloidosis, Primary Localized Cutaneous, 2
Role
preferred
Name
Amyloidosis, Primary Cutaneous
Role
preferred
Name
AMYLOIDOSIS IX
Role
alias
Name
AMYLOIDOSIS, FAMILIAL CUTANEOUS LICHEN
Role
alias
Name
AMYLOIDOSIS, PRIMARY CUTANEOUS, 1
Role
alias
Name
Amyloidosis of Skin
Role
alias
Name
Cutaneous Amyloidosis
Role
alias
Name
LICHEN AMYLOIDOSIS, FAMILIAL
Role
alias
Name
PCA
Role
alias
Name
PCA1
Role
alias
Name
PLCA1
Role
alias
Name
PLCA2
Role
alias
Name
Primary Cutaneous Amyloidosis
Role
alias

Cross References

Trusted external identifiers retained for this final record.

Hpo
HP:0012309
Herb
HBDIS006296HBDIS006297HBDIS027205HBDIS029287
Omim
105250613955
Umls
C0268397C0268398C3151404
Icd10
L99.0
Sym Map
SMDE00085SMDE05071SMDE05803
Do Class
DOID:0014667DOID:7
Dis Ge Net
C0268397C0268398C3151404C4551501
Umls Sty
T047
Hpo Class
HP:0001939
Me Sh Class
C16C17C18
Etcm Disease
Amyloidosis, Primary Localized Cutaneous, 1Amyloidosis, Primary Localized Cutaneous, 2
Tcmbank Disease
113151291613694
Itcmdb Generated
ITX-DISEASE-0C5BC89DBD2BITX-DISEASE-325441C0040A

Attributes

Merged source attributes and domain-specific metadata.

Version
v1,v2v2
Suppress
0
Page Title
Disease Amyloidosis, Primary Localized Cutaneous, 1 Details pageDisease Amyloidosis, Primary Localized Cutaneous, 2 Details page
Do Class Name
disease of metabolism; disease of anatomical entity
Disease Type
disease
Hpo Class Name
Abnormality of metabolism/homeostasis
Do Disease Class
disease of anatomical entity; disease of metabolism
Hpo Disease Class
Abnormality of metabolism/homeostasis
Umls Disease Type
Disease or Syndrome
Basic Information
Disease Name
Amyloidosis, Primary Localized Cutaneous, 1
Global Category
Genetic diseases;Metabolic diseases;Rare diseases
Anatomical Category
Skin diseases
Disease Name
Amyloidosis, Primary Localized Cutaneous, 2
Global Category
Genetic diseases;Metabolic diseases;Rare diseases
Anatomical Category
Skin diseases
Disease Definition
SNOMEDCT_US_2016_09_01:A rare chronic form of cutaneous amyloidosis, a skin disease with characteristics of the accumulation of amyloid deposits in the dermis. Clinical manifestations include the development of pruritic, often pigmented hyperkeratotic papules on trunk and extremities, especially on the shins. Histological findings include the deposition of amyloid or amyloid-like proteins in the papillary dermis.|HPO2016_07_04:The presence of amyloid deposition in the superficial dermis. [HPO:probinson, pmid:19690585]
Me Sh Disease Class
Skin and Connective Tissue Diseases; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases
Dis Ge Net Disease Type
disease
Disease Class Name Me Sh
Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Skin and Connective Tissue Diseases
Umls Semantic Type Name
Disease or Syndrome