Pityriasis Rubra Pilaris malady
Genetic diseases, Rare diseases, Skin diseases categories
Aliases & Descriptions for Pityriasis Rubra Pilaris:
MalaCards categories: See all MalaCards categories (disease lists)
Global: Genetic diseases, Rare diseases
Anatomical: Skin diseases
ICD10: 26 25
Rare skin diseases
Characteristics (Orphanet epidemiological data):48
pityriasis rubra pilaris:
Inheritance: Autosomal dominant,Not applicable; Prevalence: <1/1000000 (Worldwide); Age of onset: Infancy,Neonatal
OMIM:46 Pityriasis rubra pilaris is an uncommon skin disorder characterized by the appearance of keratotic follicular papules,... (173200) more...
MalaCards based summary: Pityriasis Rubra Pilaris, also known as familial pityriasis rubra pilaris, is related to psoriasis and scrapie, and has symptoms including palmoplantar keratoderma, irregular hyperpigmentation and pruritus. An important gene associated with Pityriasis Rubra Pilaris is CARD14 (caspase recruitment domain family, member 14). Affiliated tissues include skin, tongue and thyroid.
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.
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:64 Pityriasis rubra pilaris (also known as \"Devergie\'s disease,\" \"Lichen ruber acuminatus,\" and... more...
Symptoms by clinical synopsis from OMIM:173200
Clinical features from OMIM:173200
Symptoms:48 (show all 14)
HPO human phenotypes related to Pityriasis Rubra Pilaris:(show all 13)
MalaCards organs/tissues related to Pityriasis Rubra Pilaris:31
Skin, Tongue, Thyroid, Liver, Colon, Lung, Testes
Articles related to Pityriasis Rubra Pilaris:(show top 50) (show all 363)
UniProtKB/Swiss-Prot genetic disease variations for Pityriasis Rubra Pilaris:63
Clinvar genetic disease variations for Pityriasis Rubra Pilaris:5
Search GEO for disease gene expression data for Pityriasis Rubra Pilaris.
26ICD10 via Orphanet
34MESH via Orphanet
47OMIM via Orphanet
57SNOMED-CT via Orphanet
62UMLS via Orphanet