PRP
MCID: PTY003
MIFTS: 47

Pityriasis Rubra Pilaris (PRP) malady

Skin diseases, Genetic diseases categories

Summaries for Pityriasis Rubra Pilaris

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21Genetics Home Reference, 42NIH Rare Diseases, 63Wikipedia, 46OMIM, 32MalaCards
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Genetics Home Reference:21 Familial pityriasis rubra pilaris is a rare genetic condition that affects the skin. The name of the condition reflects its major features: The term "pityriasis" refers to scaling; "rubra" means redness; and "pilaris" suggests the involvement of hair follicles in this disorder. Affected individuals have a salmon-colored skin rash covered in fine scales. This rash occurs in patches all over the body, with distinct areas of unaffected skin between the patches. Affected individuals also develop bumps called follicular keratoses that occur around hair follicles. The skin on the palms of the hands and soles of the feet often becomes thick, hard, and callused, a condition known as palmoplantar keratoderma.

MalaCards: Pityriasis Rubra Pilaris, also known as familial pityriasis rubra pilaris, is related to psoriasis and keratosis, and has symptoms including lichen, pustula/pustulosis and dysplastic/thick/grooved fingernails. An important gene associated with Pityriasis Rubra Pilaris is CARD14 (caspase recruitment domain family, member 14). Affiliated tissues include skin, tongue and thyroid.

NIH Rare Diseases:42 Pityriasis rubra pilaris (prp) refers to a group of skin conditions that cause constant inflammation and scaling of the skin. affected people have reddish-orange colored patches; they may occur everywhere on the body or only on certain areas. there are several types of prp, which are classified based on age of onset, body areas affected, and whether other associated conditions are present. prp is usually sporadic (occurring randomly) but some forms may be inherited. last updated: 3/10/2014

Wikipedia:63 Pityriasis rubra pilaris (also known as \"Devergie\'s disease,\" \"Lichen ruber acuminatus,\" and... more...

Description from OMIM:46 173200

Aliases & Classifications for Pityriasis Rubra Pilaris

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8Disease Ontology, 9diseasecard, 42NIH Rare Diseases, 20GeneTests, 21Genetics Home Reference, 46OMIM, 10DISEASES, 48Orphanet, 60UMLS, 39NCIt, 34MeSH, 56SNOMED-CT, 27ICD9CM, 35MESH via Orphanet, 26ICD10 via Orphanet, 57SNOMED-CT via Orphanet, 61UMLS via Orphanet, 25ICD10
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Genetic diseases
Anatomical: Skin diseases


Characteristics (Orphanet epidemiological data):

48
pityriasis rubra pilaris:
Inheritance: Autosomal dominant,Sporadic; Prevalence: <1/1000000; Age of onset: Neonatal/infancy


Aliases & Descriptions:

pityriasis rubra pilaris 8 9 42 20 46 10 48 60
familial pityriasis rubra pilaris 42 21 60
pityriasis rubra pilaris--familial type 42
devergie's disease 8
familial prp 21
prp 42


External Ids:

Disease Ontology8 DOID:9212
OMIM46 173200
NCIt39 C85014
MeSH34 D010916
ICD9CM27 696.4
SNOMED-CT56 3755001
MESH via Orphanet35 D010916
ICD10 via Orphanet26 L44.0
SNOMED-CT via Orphanet57 3755001
UMLS via Orphanet61 C0032027
ICD1025 L44.0

Related Diseases for Pityriasis Rubra Pilaris

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17GeneCards, 18GeneDecks
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Graphical network of the top 20 diseases related to Pityriasis Rubra Pilaris:



Diseases related to pityriasis rubra pilaris

Clinical Features for Pityriasis Rubra Pilaris

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46OMIM, 48Orphanet
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Clinical features from OMIM:

173200

Clinical synopsis from OMIM:

173200

Symptoms:

48 (show all 14)
  • lichen
  • pustula/pustulosis
  • dysplastic/thick/grooved fingernails
  • eczema
  • neoplasms/tumors
  • nails anomalies
  • erythema/erythematous lesions/erythroderma/polymorphous erythema
  • follicular/erythematous/edematous papules/milium
  • pruritus/itching
  • irregular/in bands/reticular skin hyperpigmentation
  • palmoplantar hyperkeratosis/keratoderma
  • anomalies of tongue, gingiva and oral mucosa
  • thick skin/pachydermia/orange skin
  • ichthyosis/ichthyosiform dermatitis

Drugs & Therapeutics for Pityriasis Rubra Pilaris

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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Pityriasis Rubra Pilaris

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20GeneTests
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Genetic tests related to Pityriasis Rubra Pilaris:

id Genetic test Affiliating Genes
1 Pityriasis Rubra Pilaris20 CARD14

Anatomical Context for Pityriasis Rubra Pilaris

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32MalaCards
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MalaCards organs/tissues related to Pityriasis Rubra Pilaris:

32
Skin, Tongue, Thyroid, Liver, Testes, Colon, Lung

Animal Models for Pityriasis Rubra Pilaris or affiliated genes

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Publications for Pityriasis Rubra Pilaris

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50PubMed
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Articles related to Pityriasis Rubra Pilaris:

(show top 50)    (show all 347)
idTitleAuthorsYear
1
Drug-related pityriasis rubra pilaris with acantholysis. (24266317)
2013
2
Pityriasis rubra pilaris: was it the first manifestation of colon cancer in a patient with pre-existing psoriasis? (23317984)
2013
3
Pityriasis rubra pilaris treated with methotrexate resolving with an erythema gyratum repens-like appearance. (23768305)
2013
4
Long-term ustekinumab treatment for refractory type I pityriasis rubra pilaris. (23580907)
2013
5
Familial pityriasis rubra pilaris is caused by mutations in CARD14. (22703878)
2012
6
Papules and plaques from head to foot. Pityriasis rubra pilaris. (22991881)
2012
7
Successful treatment with infliximab despite positive tuberculosis ELISpot results in a patient with pityriasis rubra pilaris taking prophylactic isoniazid. (21507042)
2011
8
Pityriasis rubra pilaris with prominent acantholytic dyskeratosis. (21719401)
2011
9
Treatment of refractory adult-onset pityriasis rubra pilaris with TNF-alpha antagonists: a case series. (20002243)
2010
10
Successful treatment of type 1 pityriasis rubra pilaris with ustekinumab therapy. (20605773)
2010
11
Concomitant severe Kawasaki disease and pityriasis rubra pilaris and in a teenager: just a coincidence? (20412717)
2010
12
Clinical improvement of pityriasis rubra pilaris with combination etanercept and acitretin therapy. (18087024)
2007
13
Treatment of pityriasis rubra pilaris with pimecrolimus cream 1%. (17373199)
2007
14
(Juvenile) Pityriasis rubra pilaris. (16650174)
2006
15
Pityriasis rubra pilaris: a clinical review. (16463927)
2005
16
Pityriasis rubra pilaris, type IV. (16403386)
2005
17
Paraneoplastic pityriasis rubra pilaris in association with bronchogenic carcinoma. (15068463)
2004
18
Juvenile pityriasis rubra pilaris. (15283808)
2004
19
Classical juvenile pityriasis rubra pilaris in a patient with Down syndrome. (14723722)
2004
20
Merkel cell carcinoma and multiple cutaneous squamous cell carcinomas in a patient with pityriasis rubra pilaris. (11869209)
2002
21
Three cases of pityriasis rubra pilaris successfully treated with cyclosporin A. (10894965)
2000
22
Bath-PUVA as a treatment for Pityriasis rubra pilaris provoked by ultraviolet B. (10233350)
1999
23
The relationship between pityriasis rubra pilaris and inflammatory arthritis: case report and response of the arthritis to anti-tumor necrosis factor immunotherapy. (10513817)
1999
24
Lymphocyte response to superantigen in a patient with childhood-onset pityriasis rubra pilaris. (10487460)
1999
25
Narrow-band UVB-associated lesional blisters in pityriasis rubra pilaris. (10534654)
1999
26
Pneumocystis carinii pneumonia complicating methotrexate treatment of pityriasis rubra pilaris. (9704846)
1998
27
Early presentation of pityriasis rubra pilaris. (9426969)
1997
28
Extensive extraspinal hyperostoses after long-term oral retinoid treatment in a patient with pityriasis rubra pilaris. (7829733)
1995
29
Pityriasis rubra pilaris presenting as subacute cutaneous lupus erythematosus. (8243104)
1993
30
Pityriasis rubra pilaris in a patient with human immunodeficiency virus infection. (1430367)
1992
31
Pityriasis rubra pilaris in a child seropositive for the human immunodeficiency virus. (1479079)
1992
32
Pityriasis rubra pilaris and HIV infection. (1869640)
1991
33
Eruptive seborrheic keratoses associated with erythrodermic pityriasis rubra pilaris. Possible role of retinoid therapy. (2461032)
1988
34
Pityriasis rubra pilaris and vitiligo in Down's syndrome. (2978467)
1988
35
Pityriasis rubra pilaris. (6465925)
1984
36
Vitamin A and pityriasis rubra pilaris. (7142464)
1982
37
Retinol binding protein and pityriasis rubra pilaris. (7201847)
1982
38
The effect of 13-cis retinoic acid on epidermal lysosomal hydrolase activity in Darier's disease and pityriasis rubra pilaris. (6997395)
1980
39
Pityriasis rubra pilaris [proceedings]. (159266)
1979
40
Pityriasis rubra pilaris epidermal cells in vitro. A comparison with normal and psoriatic cells. (603257)
1977
41
Pityriasis rubra pilaris in Korea. Treatment with methotrexate. (5371703)
1969
42
Oral lesions in pityriasis rubra pilaris. (5239091)
1968
43
Pityriasis rubra pilaris responsive to ascorbic acid. (5673924)
1968
44
Vitamin A--responsive pityriasis rubra pilaris with myasthenia gravis. (5835336)
1965
45
Pityriasis rubra pilaris; refractoriness to vitamin A therapy. (18098653)
1948
46
A case for diagnosis (pityriasis rubra pilaris in one of twin brothers?). (21065255)
1946
47
Pityriasis rubra pilaris. (20983403)
1946
48
Pityriasis Rubra Pilaris. Case for Diagnosis. (19986546)
1928
49
Case of Pityriasis Rubra Pilaris. (19984906)
1926
50
Pityriasis Rubra Pilaris in a Girl, aged 5. (19975055)
1911

Genetic Variations for Pityriasis Rubra Pilaris

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62UniProtKB/Swiss-Prot
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Genetic disease variations for Pityriasis Rubra Pilaris:

62
id Symbol AA change Variation ID SNP ID
1CARD14p.Leu156ProVAR_068820

Expression for genes affiliated with Pityriasis Rubra Pilaris

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Pityriasis Rubra Pilaris

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Pathways for genes affiliated with Pityriasis Rubra Pilaris

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Compounds for genes affiliated with Pityriasis Rubra Pilaris

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GO Terms for genes affiliated with Pityriasis Rubra Pilaris

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Products for genes affiliated with Pityriasis Rubra Pilaris

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  • Antibodies
  • Proteins
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Sources for Pityriasis Rubra Pilaris

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet