PRP
MCID: PTY003
MIFTS: 41

Pityriasis Rubra Pilaris (PRP) malady

Skin, Genetic categories

Summaries for Pityriasis Rubra Pilaris

Sources:
21Genetics Home Reference, 43NIH Rare Diseases, 64Wikipedia, 47OMIM, 33MalaCards
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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 scrapie and bovine spongiform encephalopathy, and has symptoms including ichthyosis/ichthyosiform dermatitis, nails anomalies and erythema/erythematous lesions/erythroderma/polymorphous erythema. An important gene associated with Pityriasis Rubra Pilaris is CARD14 (caspase recruitment domain family, member 14). Affiliated tissues include skin, liver and lung.

NIH Rare Diseases:43 Pityriasis rubra pilaris is a skin disorder that causes constant inflammation and scaling (exfoliation) of the skin. pityriasis rubra pilaris can be divided into 5 categories: classic adult type, atypical adult type, classic juvenile type, circumscribed juvenile type, and atypical juvenile type. more recently, an hiv-associated type has been added to this classification system. this condition can be inherited or acquired. pityriasis rubra pilaris may occur at any age, with the familial type commonly starting in early childhood and the acquired type in the fifth or sixth decades of life. both men and women are affected equally. last updated: 6/30/2011

Wikipedia:64 Pityriasis rubra pilaris (also known as \"Devergie\'s disease,\" \"Lichen ruber acuminatus,\" and... more...

Description from OMIM:47 173200

Aliases & Classifications for Pityriasis Rubra Pilaris

Sources:
8Disease Ontology, 9diseasecard, 43NIH Rare Diseases, 20GeneTests, 21Genetics Home Reference, 47OMIM, 10DISEASES, 49Orphanet, 61UMLS, 40NCIt, 35MeSH, 57SNOMED-CT, 27ICD9CM, 36MESH via Orphanet, 26ICD10 via Orphanet, 58SNOMED-CT via Orphanet, 62UMLS via Orphanet, 25ICD10
See all sources

Classifications:

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


Characteristics (Orphanet epidemiological data):

49
pityriasis rubra pilaris:
Inheritance: Autosomal dominant,Sporadic; Prevalence: <1/1000000; Age of onset: Neonatal/infancy


Aliases & Descriptions:

pityriasis rubra pilaris 8 9 43 20 47 10 49 61
familial pityriasis rubra pilaris 43 21 61
pityriasis rubra pilaris--familial type 43
devergie's disease 8
familial prp 21
prp 43


External Ids:

Disease Ontology8 DOID:9212
OMIM47 173200
NCIt40 C85014
MeSH35 D010916
ICD9CM27 696.4
SNOMED-CT57 3755001
MESH via Orphanet36 D010916
ICD10 via Orphanet26 L44.0
SNOMED-CT via Orphanet58 3755001
UMLS via Orphanet62 C0032027
ICD1025 L44.0

Related Diseases for Pityriasis Rubra Pilaris

Sources:
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

Sources:
47OMIM, 49Orphanet
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Clinical features from OMIM:

173200

Clinical synopsis from OMIM:

173200

Symptoms:

49 (show all 14)
  • ichthyosis/ichthyosiform dermatitis
  • 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
  • neoplasms/tumors
  • eczema
  • dysplastic/thick/grooved fingernails
  • pustula/pustulosis
  • lichen

Drugs & Therapeutics for Pityriasis Rubra Pilaris

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

Search CenterWatch for Pityriasis Rubra Pilaris

Drug clinical trials:

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Search NIH Clinical Center for Pityriasis Rubra Pilaris

Search CenterWatch for Pityriasis Rubra Pilaris

Genetic Tests for Pityriasis Rubra Pilaris

Sources:
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

Sources:
33MalaCards
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MalaCards organs/tissues related to Pityriasis Rubra Pilaris:

33
Skin, Liver, Lung, Colon, Thyroid, B cells

Animal Models for Pityriasis Rubra Pilaris or affiliated genes

Sources:
28inGenious Targeting Laboratory
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Publications for Pityriasis Rubra Pilaris

Sources:
51PubMed
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Articles related to Pityriasis Rubra Pilaris:

(show top 50)    (show all 347)
idTitleAuthorsYear
1
Pityriasis rubra pilaris-like reaction induced by imatinib. (23777493)
2013
2
Etanercept-induced clinical remission of type II pityriasis rubra pilaris with rheumatoid arthritis. (22513698)
2012
3
Oral alitretinoin for the treatment of recalcitrant pityriasis rubra pilaris. (21577368)
2011
4
Should ustekinumab really be used as first-line biological therapy in pityriasis rubra pilaris? (20662839)
2010
5
Penicillin and vitamin A as possible therapeutic agents in pityriasis rubra pilaris. (19912462)
2010
6
Type I pityriasis rubra pilaris: upregulation of tumor necrosis factor alpha and response to adalimumab therapy. (20642989)
2010
7
Pityriasis rubra pilaris sensitive to narrow band-ultraviolet B light therapy. (19271375)
2009
8
Eruptive seborrhoeic keratoses associated with erythrodermic pityriasis rubra pilaris. (18482315)
2009
9
Treatment of juvenile pityriasis rubra pilaris with etanercept. (19119116)
2008
10
Photo quiz. Juvenile pityriasis rubra pilaris. (17674582)
2007
11
Pityriasis rubra pilaris in a mother and two daughters. (17489979)
2007
12
Successful treatment of type I adult-onset pityriasis rubra pilaris with infliximab. (16637810)
2006
13
Living with pityriasis rubra pilaris. (16294947)
2005
14
Pityriasis rubra pilaris in children. (12196748)
2002
15
Atypical pityriasis rubra pilaris associated with arthropathy and osteoporosis: a case report with 15-year follow-up. (11860571)
2002
16
Combined ultraviolet A1 radiation and acitretin therapy as a treatment option for pityriasis rubra pilaris. (10777268)
2000
17
Pityriasis rubra pilaris in black South Africans. (10354168)
1999
18
Arthropathic pityriasis rubra pilaris. (8763450)
1996
19
Pityriasis rubra pilaris and human immunodeficiency virus infection--type 6 pityriasis rubra pilaris? (8983330)
1996
20
Picture of the month. Juvenile pityriasis rubra pilaris. (7633550)
1995
21
Pityriasis rubra pilaris as the initial manifestation of internal neoplasia. (8593728)
1995
22
Topical treatment of pityriasis rubra pilaris with calcipotriol. (7515639)
1994
23
Pityriasis rubra pilaris with leprophobia. (3655467)
1987
24
Childhood-onset pityriasis rubra pilaris with immunologic abnormalities. (2954034)
1987
25
Pityriasis rubra pilaris, vitamin A and retinol-binding protein: a case study. (6208725)
1984
26
Pityriasis rubra pilaris as the initial manifestation of leukemia. (6825457)
1983
27
Pityriasis rubra pilaris--an autoradiographic study. (7171485)
1982
28
Pityriasis rubra pilaris response to 13-cis-retinoic acid (isotretinoin). (7040513)
1982
29
Evaluation of 13-cis retinoic acid in lamellar ichthyosis, pityriasis rubra pilaris and Darier's disease. (6928816)
1980
30
Pityriasis rubra pilaris and celiac sprue with malabsorption. (7379593)
1980
31
Pityriasis rubra pilaris--an historical approach. 2. Clinical features. (1269179)
1976
32
Case for diagnosis: pityriasis rubra pilaris? (4649383)
1972
33
Treatment of pityriasis rubra pilaris with azathioprine. (5043197)
1972
34
Pityriasis rubra pilaris. (4254887)
1971
35
Epidermal renewal and amino acids in psoriasis and pityriasis rubra pilaris. (5676686)
1968
36
Pityriasis rubra pilaris treated with methotrexate. (5964595)
1966
37
Brief clinical reports of two cases of pityriasis rubra pilaris. (13793813)
1960
38
Pityriasis rubra pilaris in identical twins. (13499819)
1958
39
Conversion of carotene to vitamin A by sebaceous glands; interference in pityriasis rubra pilaris, ichthyosis, and psoriasis as shown by fluorescent microscopy. (13457425)
1957
40
Pityriasis rubra pilaris; clinical and laboratory observations on combined treatment with corticotropin and vitamin A. (14922956)
1952
41
Pityriasis rubra pilaris. (20342290)
1947
42
Pityriasis Rubra Pilaris with Ainhum and Syringomyelia. (19993248)
1946
43
Pityriasis rubra pilaris. (20995066)
1946
44
Pityriasis Rubra Pilaris. (19989256)
1933
45
Pityriasis Rubra Pilaris. Case for Diagnosis. (19988617)
1932
46
Two Cases of (?) Pityriasis Rubra Pilaris. (19981657)
1921
47
Case of Pityriasis Rubra Pilaris. (19981610)
1921
48
Pityriasis Rubra Pilaris (Generalized Form). (19980885)
1920
49
Case of Pityriasis Rubra Pilaris. (19979111)
1916
50
Psoriasis and Pityriasis Rubra Pilaris. (19976645)
1913

Genetic Variations for Pityriasis Rubra Pilaris

Sources:
63UniProtKB/Swiss-Prot
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Genetic disease variations for Pityriasis Rubra Pilaris:

63
id Symbol AA change Variation SNP ID
1CARD14p.Leu156ProVAR_068820

Expression for genes affiliated with Pityriasis Rubra Pilaris

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

Search GEO for disease gene expression data for Pityriasis Rubra Pilaris.

Pathways for genes affiliated with Pityriasis Rubra Pilaris

Compounds for genes affiliated with Pityriasis Rubra Pilaris

GO Terms for genes affiliated with Pityriasis Rubra Pilaris

Products for genes affiliated with Pityriasis Rubra Pilaris

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Sources for Pityriasis Rubra Pilaris

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet