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 neoplasms/tumors, pustula/pustulosis and lichen. 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, 46OMIM, 10DISEASES, 48Orphanet, 60UMLS, 21Genetics Home Reference, 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)
  • neoplasms/tumors
  • pustula/pustulosis
  • lichen
  • eczema
  • ichthyosis/ichthyosiform dermatitis
  • erythema/erythematous lesions/erythroderma/polymorphous erythema
  • follicular/erythematous/edematous papules/milium
  • irregular/in bands/reticular skin hyperpigmentation
  • pruritus/itching
  • thick skin/pachydermia/orange skin
  • nails anomalies
  • dysplastic/thick/grooved fingernails
  • anomalies of tongue, gingiva and oral mucosa
  • palmoplantar hyperkeratosis/keratoderma

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, Lung, Testes, Colon

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
Effectiveness of infliximab in pityriasis rubra pilaris is associated with pro-inflammatory cytokine inhibition. (23548788)
2013
2
Pityriasis rubra pilaris types 1 and 2: different responses to treatment with TNF-alpha antagonists. (24192269)
2013
3
Adalimumab for the treatment of pityriasis rubra pilaris: a case report. (23270195)
2012
4
Pityriasis rubra pilaris. (23286804)
2012
5
Telaprevir-induced pityriasis rubra pilaris-like drug eruption. (23069976)
2012
6
Pityriasis rubra pilaris: the clinical context of acantholysis and other histologic features. (22097993)
2011
7
Familial pityriasis rubra pilaris: report of a family and therapeutic response to etanercept. (20677542)
2010
8
Familial combined hypercholesterolemia type II b presenting with tuberous xanthoma, tendinous xanthoma and pityriasis rubra pilaris-like lesions. (20445310)
2010
9
Pityriasis rubra pilaris in association with laryngeal carcinoma. (20055867)
2009
10
Pityriasis rubra pilaris responding rapidly to adalimumab. (19153364)
2009
11
A case of juvenile pityriasis rubra pilaris: could varicella be an aetiological agent? (20055822)
2009
12
Pityriasis rubra pilaris in a HIV-positive patient (Type 6 PRP). (18174806)
2008
13
Pityriasis rubra pilaris successfully treated with infliximab. (17989902)
2007
14
Type II adult-onset pityriasis rubra pilaris successfully treated with intravenous immunoglobulin. (17313492)
2007
15
Juvenile onset classical pityriasis rubra pilaris: every patient may not require systemic therapy. (17903408)
2007
16
Photosensitive pityriasis rubra pilaris. (16309476)
2006
17
Oral pityriasis rubra pilaris. (16632271)
2006
18
Pityriasis rubra pilaris. (16463930)
2005
19
Pityriasis rubra pilaris. (12768049)
2003
20
Familial pityriasis rubra pilaris (adult classic-I): a report of three cases in a single family. (14673347)
2002
21
Phototesting and phototherapy in pityriasis rubra pilaris. (11251596)
2001
22
Pityriasis rubra pilaris treated with acitretin and narrow-band ultraviolet B (Re-TL-01) (10730782)
2000
23
Pityriasis rubra pilaris with acantholysis and lichenoid histology. (10535583)
1999
24
Familial pityriasis rubra pilaris. (7726588)
1995
25
Pityriasis rubra pilaris and cyclosporine. (8215510)
1993
26
Horny perifollicular mucinosis. An atypical pityriasis rubra pilaris-like eruption associated with HIV infection. (8214392)
1993
27
Pityriasis rubra pilaris: the problem of its classification. (1732330)
1992
28
Pityriasis rubra pilaris: the problem of its classification. (2091649)
1990
29
Pityriasis rubra pilaris. Further observations of systemic retinoid therapy. (2347965)
1990
30
Focal acantholytic dyskeratosis occurring in pityriasis rubra pilaris. (2316811)
1990
31
Dramatic follicular plugging in pityriasis rubra pilaris. (2212163)
1990
32
Pityriasis rubra pilaris in childhood: a long-term study of 29 cases. (3562357)
1986
33
The nails in adult type 1 pityriasis rubra pilaris. A comparison with SAczary syndrome and psoriasis. (3782535)
1986
34
Serum levels of retinol binding protein in patients with pityriasis rubra pilaris. (6681980)
1983
35
Pityriasis rubra pilaris. (7398119)
1980
36
Toxic doses of vitamin A for pityriasis rubra pilaris. (7406516)
1980
37
Pityriasis rubra pilaris responding to aminonicotinamide. (150817)
1978
38
Pityriasis rubra pilaris. A clinico-pathological study with a special reference to autoradiography and histocompatibility antigens. (939336)
1976
39
Topical administration of vitamin A acid (retinoic acid) in palmar keratoses: callosities, hyperkeratotic eczema, hypertrophic lichen planus, pityriasis rubra pilaris. (4569794)
1972
40
Pityriasis rubra pilaris with stange localization; report of a case. (13544629)
1958
41
A case of pityriasis rubra pilaris. (13401290)
1957
42
Pityriasis rubra pilaris with ainhum and syringomyelia. (21024123)
1946
43
Pityriasis rubra pilaris. (21064487)
1946
44
Pityriasis Rubra Pilaris. (19990958)
1937
45
Pityriasis Rubra Pilaris. (19990254)
1935
46
Psoriasis. ? Pityriasis Rubra Pilaris. Case for Diagnosis. (19987354)
1930
47
Case of Pityriasis Rubra Pilaris. (19984889)
1926
48
Case of Pityriasis Rubra Pilaris. (19984882)
1926
49
Case of Pityriasis Rubra Pilaris. (19984265)
1925
50
Case of Pityriasis Rubra Pilaris. (19980925)
1920

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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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