Condition

Pityriasis rubra pilaris

Diseases of the Skin and Subcutaneous Tissue

Pityriasis rubra pilaris, often shortened to PRP, refers to a group of chronic disorders marked by reddish orange scaling plaques and keratotic follicular papules. The name carries its description: rubra for the reddish patches, pityriasis for the severe flaking, and pilaris from the Latin for hair, for the thickened bumps around hair follicles. Symptoms can also include itching, thickened skin on the hands and feet, and generalized swelling of the legs and feet. Its course and rate of improvement vary, and the source says there is currently no known cause or cure. It was first described by Marie-Guillaume-Alphonse Devergie in 1856 and is also called Devergie's disease. This record is not medical advice.

Facts
Classification
Body System
Integumentary System 1
ICD-10 Chapter
XII: Diseases of the Skin and Subcutaneous Tissue 2
First Described
1856 1
Prevalence Band
Rare (Under 1 in 2000) 4
Causative Agent
Causative Agent (category)
Idiopathic / Multifactorial 3
Sources
1. Pityriasis rubra pilaris (Wikipedia)
  • Opening sentence
    reddish orange, scaling plaques and keratotic follicular papules
  • Lead, second paragraph
    first described by Marie-Guillaume-Alphonse Devergie in 1856
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2. World Health Organization: ICD-10 Version:2019 - L44.0
World Health OrganizationICD-10 2019 category L44.0
Quote, ICD-10 2019 category L44.0
L44.0 Pityriasis rubra pilaris
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3. National Cancer Institute (NCI Thesaurus): Pityriasis rubra pilaris - NCI Thesaurus concept C85014
National Cancer Institute (NCI Thesaurus)Definition
Quote, Definition
A rare cutaneous disorder of unknown etiology characterized by the presence of reddish-orange scaly plaques, palmoplantar keratoderma, keratotic follicular papules, and confluent erythematous areas with areas of normal skin between the lesions.
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4. Orphadata (Orphanet): Orphadata epidemiology, ORPHAcode 2897
Orphadata (Orphanet)Orphanet epidemiology, ORPHAcode 2897
Quote, Orphanet epidemiology, ORPHAcode 2897
"PrevalenceClass": "1-9 / 1 000 000", "PrevalenceGeographic": "Worldwide", "PrevalenceQualification": "Class only", "PrevalenceType": "Point prevalence"
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