Raras
Buscar doenças, sintomas, genes...
Pytiriasis rubra pilosa
ORPHA:2897CID-10 · L44.0CID-11 · EA94OMIM 173200DOENÇA RARA

Doença papuloescamosa crônica rara, de etiologia desconhecida, caracterizada por pequenas pápulas foliculares, manchas vermelho-alaranjadas escamosas e hiperqueratose palmoplantar, que pode progredir para placas ou eritrodermia. Embora a maioria dos casos seja esporádica e adquirida, existe uma forma familiar da doença.

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Introdução

O que você precisa saber de cara

📋

Doença papuloescamosa crônica rara, de etiologia desconhecida, caracterizada por pequenas pápulas foliculares, manchas vermelho-alaranjadas escamosas e hiperqueratose palmoplantar, que pode progredir para placas ou eritrodermia. Embora a maioria dos casos seja esporádica e adquirida, existe uma forma familiar da doença.

Pesquisas ativas
2 ensaios
6 total registrados no ClinicalTrials.gov
Publicações científicas
1.008 artigos
Último publicado: 2026 Apr

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Casos conhecidos
48
pacientes catalogados
Início
All ages
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: L44.0
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Características mais comuns

90%prev.
Pápula
Muito frequente (99-80%)
90%prev.
Ceratodermia palmoplantar
Muito frequente (99-80%)
90%prev.
Eritrodermia
Muito frequente (99-80%)
90%prev.
Hiperpigmentação irregular
Muito frequente (99-80%)
55%prev.
Hiperceratose subungueal
Frequente (79-30%)
55%prev.
Prurido
Frequente (79-30%)
22sintomas
Muito frequente (4)
Frequente (4)
Ocasional (7)
Sem dados (7)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 22 características clínicas mais associadas, ordenadas por frequência.

PápulaPapule
Muito frequente (99-80%)90%
Ceratodermia palmoplantarPalmoplantar keratoderma
Muito frequente (99-80%)90%
EritrodermiaErythroderma
Muito frequente (99-80%)90%
Hiperpigmentação irregularIrregular hyperpigmentation
Muito frequente (99-80%)90%
Hiperceratose subunguealSubungual hyperkeratosis
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico1.008PubMed
Últimos 10 anos200publicações
Pico202458 papers
Linha do tempo
2026Hoje · 2026🧪 2008Primeiro ensaio clínico📈 2024Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

1 gene identificado com associação a esta condição. Padrão de herança: Autosomal dominant, Not applicable.

CARD14Caspase recruitment domain-containing protein 14Disease-causing germline mutation(s) inTolerante
FUNÇÃO

Acts as a scaffolding protein that can activate the inflammatory transcription factor NF-kappa-B and p38/JNK MAP kinase signaling pathways. Forms a signaling complex with BCL10 and MALT1, and activates MALT1 proteolytic activity and inflammatory gene expression. MALT1 is indispensable for CARD14-induced activation of NF-kappa-B and p38/JNK MAP kinases (PubMed:11278692, PubMed:21302310, PubMed:27071417, PubMed:27113748). May play a role in signaling mediated by TRAF2, TRAF3 and TRAF6 and protects

LOCALIZAÇÃO

Cytoplasm

MECANISMO DE DOENÇA

Psoriasis 2

A common, chronic inflammatory disease of the skin with multifactorial etiology. It is characterized by red, scaly plaques usually found on the scalp, elbows and knees. These lesions are caused by abnormal keratinocyte proliferation and infiltration of inflammatory cells into the dermis and epidermis.

OUTRAS DOENÇAS (2)
familial pityriasis rubra pilarispsoriasis 2
HGNC:16446UniProt:Q9BXL6

Medicamentos aprovados (FDA)

1 medicamento encontrado nos registros da FDA americana.

💊 Salicylic Acid 6% Gel (SALICYLIC ACID)
Ver no DailyMed/FDA

Variantes genéticas (ClinVar)

82 variantes patogênicas registradas no ClinVar.

🧬 CARD14: NM_001366385.1(CARD14):c.357G>T (p.Met119Ile) ()
🧬 CARD14: NM_001366385.1(CARD14):c.1570del (p.Asp524fs) ()
🧬 CARD14: NM_001366385.1(CARD14):c.722C>T (p.Ser241Phe) ()
🧬 CARD14: NM_001366385.1(CARD14):c.1824dup (p.Arg610fs) ()
🧬 CARD14: NM_001366385.1(CARD14):c.*19G>A ()
Ver todas no ClinVar

Classificação de variantes (ClinVar)

Distribuição de 1,236 variantes classificadas pelo ClinVar.

742
494
VUS (60.0%)
Benigna (40.0%)
VARIANTES MAIS SIGNIFICATIVAS
CARD14: NM_001366385.1(CARD14):c.2822G>T (p.Arg941Leu) [Uncertain significance]
CARD14: NM_001366385.1(CARD14):c.3001C>T (p.Gln1001Ter) [Uncertain significance]
CARD14: NM_001366385.1(CARD14):c.1586A>G (p.Asp529Gly) [Uncertain significance]
CARD14: NM_001366385.1(CARD14):c.2951G>A (p.Cys984Tyr) [Uncertain significance]
CARD14: NM_001366385.1(CARD14):c.455G>A (p.Arg152Gln) [Uncertain significance]

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
Aprovado2
2Fase 23
1Fase 11
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 6 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Pytiriasis rubra pilosa

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

1 pesquisa recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

6 ensaios clínicos encontrados, 2 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
448 papers (10 anos)
#1

Successful Treatment of Pityriasis Rubra Pilaris With Deucravacitinib.

JAMA dermatology2026 Mar 01

This case report describes a woman in her 60s who presented with a 2-month history of rapidly progressive erythematous skin lesions that began on the upper extremities and gradually spread to the buttocks and lower limbs and was diagnosed with pityriasis rubra pilaris.

#2

CARD14-Associated Papulosquamous Eruption (CAPE): An Updated Review of Pathogenesis and Treatment.

International journal of dermatology2026 Mar 17

CARD14-associated papulosquamous eruption (CAPE) is a rare genetically mediated inflammatory skin disorder caused by gain-of-function variants in the CARD14 gene. It typically presents in infancy or early childhood with clinical features resembling both psoriasis and pityriasis rubra pilaris, and often exhibits a poor response to conventional therapies. An increasing number of studies support favorable outcomes with biologic medications, particularly interleukin (IL)-12/23 and IL-17 inhibitors, for the treatment of CAPE. This literature review summarizes the etiology and pathophysiology of CAPE, with a focus on clinical features, genetic findings, and developments in treatment strategies.

#3

Case Report: Successful treatment of pityriasis rubra pilaris with deucravacitinib.

Frontiers in immunology2026

Pityriasis rubra pilaris (PRP) is a rare inflammatory skin disease characterized by hyperkeratotic follicular papules, palmoplantar hyperkeratosis, and associated normal "islands of unaffected skin". Its pathogenesis has not been fully elucidated, and treatment poses significant challenges. Conventional therapies include oral retinoids and topical emollients. In recent years, although biological agents have been used in treatment, they are associated with side effects such as an increased risk of infection, and some patients show no response to treatment, thus necessitating the exploration of new therapeutic approaches.This case represents the first reported use of a TYK2 inhibitor (deucravacitinib) for the treatment of PRP. The patient was a 39-year-old male who developed extensive erythema in December 2024. The erythema gradually increased and progressed to red punctate eruptions accompanied by mild desquamation, slight pruritus with a stinging sensation, and "islands of unaffected skin". Initial treatment with topical dinoprostone and mometasone furoate cream was ineffective. In March 2025, the patient received deucravacitinib (6 mg daily) in combination with topical halometasone cream. At the 1-month follow-up, significant improvements were observed in erythema, desquamation, and pruritus. At the final 6-month follow-up, the skin lesions almost resolved, leaving only mild erythema and a small amount of desquamation. Both the disease severity and the patient's quality of life were significantly improved. This case suggests that deucravacitinib exhibits favorable efficacy and safety in the treatment of PRP. However, due to the low incidence of PRP, which makes large-scale controlled trials difficult, and the lack of recognized treatment guidelines, more clinical studies are needed in the future to further verify the potential of deucravacitinib in the treatment of PRP.

#4

Vitamins and the skin: Vitamin A and retinoids in dermatology.

Clinics in dermatology2026 Feb 13

Vitamin A and analogs are widely used in dermatology, with retinoids being natural and synthetic vitamin A derivatives. Topical retinoids (especially tretinoin and tretinoin precursors) can diminish photoaging and contribute to the thickening and restoration of skin collagen. Retinoids used as therapeutic agents include oral retinoids (eg, isotretinoin, acitretin, alitretinoin, and bexarotene) and topical retinoids (eg, isotretinoin, tretinoin, adapalene, tazarotene, and trifarotene). Although retinoids have traditionally been used for skin disorders of keratinization, such as psoriasis, pityriasis rubra pilaris, Darier disease, and ichthyoses, there is a variety of indications of retinoids for the treatment of skin diseases, including diseases of the pilosebaceous unit such as acne vulgaris, pigmentary disorders such as melasma, or cutaneous malignancies such as cutaneous T-cell lymphoma. Other retinoids with distinct routes of administration (oral or topical) and distinct dosing or safety profiles are recommended for different skin disorders. We discuss the mode of action and indications of retinoids used as pharmacologic agents in dermatology and provide an update on their use, effectiveness, and tolerability.

#5

Pityriasis rubra pilaris triggered by vaccination.

Italian journal of dermatology and venereology2026 Feb

Pityriasis rubra pilaris (PRP) is a rare inflammatory papulo-squamous skin disease. To date, the exact etiopathogenesis of PRP is unknown; although the most accepted triggers are viral or bacterial infections, few cases after vaccination have been reported as well. Our aim is to conduct a systematic review of cases of PRP triggered by vaccination. The PubMed and Scopus databases were searched for articles concerning PRP post-vaccination, published between January 2000 and June 2024. We also added a previously unpublished case that came to our attention. Twenty-three articles were included, and 30 cases have been identified. The majority of patients were male (20/30, 66.6%). The median age of onset was 55 years (min 17 months-max 85 years). Most patients (27/30, 90%) were adults vaccinated against SARS-CoV-2, of whom 14/27 (51.9%) received mRNA-based vaccines (9 Comirnaty/Pfizer and 5 Spikevax/Moderna). The three pediatric cases had been vaccinated against Measles-Mumps-Rubella (2 cases) and intramuscular diphtheria-tetanus-pertussis vaccine plus oral poliovirus. The temporal relationship between vaccination and PRP onset varied (median 10 days post-vaccination; min 2-max 30). PRP occurred both after the first dose (14/30, 46.6%) and at subsequent doses of the vaccine. The majority of patients re-exposed to new doses (6/9, 66%) experienced clinical exacerbation. Post-vaccination PRP responds well to both traditional and biologic treatments, with only 4/30 (13.3%) showing no resolution. In conclusion, PRP post-vaccination is rare and likely underdiagnosed, but recognizing the association is important to evaluate any new exposures to the trigger. A thorough patient history, including recent vaccinations, is crucial.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC800 artigos no totalmostrando 195

2026

CARD14-Associated Papulosquamous Eruption (CAPE): An Updated Review of Pathogenesis and Treatment.

International journal of dermatology
2026

Tyrosine Kinase 2 Inhibition for Refractory Pityriasis Rubra Pilaris in Two Cases.

International journal of dermatology
2026

Familial CARD14-associated papulosquamous eruption with a novel mutation successfully treated with secukinumab.

JAAD case reports
2026

Vaccination-Induced Pityriasis Rubra Pilaris: A Systematic Review.

Journal of cutaneous medicine and surgery
2026

Case Report: Successful treatment of pityriasis rubra pilaris with deucravacitinib.

Frontiers in immunology
2026

Vitamins and the skin: Vitamin A and retinoids in dermatology.

Clinics in dermatology
2026

Successful Treatment of Pityriasis Rubra Pilaris With Deucravacitinib.

JAMA dermatology
2026

Type IV pityriasis rubra pilaris treated with ixekizumab.

Anais brasileiros de dermatologia
2025

Navigating the Misdiagnosis of Pityriasis Rubra Pilaris and Successful Treatment With Guselkumab: A Case Report of Dual Biologic Therapy.

Cureus
2025

Striking linear presentation of type I pityriasis rubra pilaris in a young man.

Clinical and experimental dermatology
2026

Pityriasis rubra pilaris triggered by vaccination.

Italian journal of dermatology and venereology
2025

Pityriasis Rubra Pilaris With Hypereosinophilia: Answer.

The American Journal of dermatopathology
2025

Cytokine-associated pathogenic profile as a guide for targeted therapy in pityriasis rubra pilaris.

Journal of the European Academy of Dermatology and Venereology : JEADV
2025

Psoriasis in the Context of Dermatologic Disorders: A Comprehensive Overview.

Diseases (Basel, Switzerland)
2026

Paraneoplastic pityriasis rubra pilaris: a systematic review.

Clinical and experimental dermatology
2025

HIV-Associated Pityriasis Rubra Pilaris Type 6: First Case Report from Türkiye.

Infectious diseases &amp; clinical microbiology
2025

Tofacitinib: A Promising Therapeutic Option for Refractory Pityriasis Rubra Pilaris-A Case Report.

Clinical case reports
2025

Regressed anterior mediastinal nodule secondary to removal of dental crowns.

Journal of surgical case reports
2025

Diagnosis and Assessment of Psoriasis for the Rheumatologist: A Workshop From the GRAPPA 2024 Annual Meeting.

The Journal of rheumatology
2025

Pediatric Pityriasis Rubra Pilaris Treated With Secukinumab: A Case Report.

The Journal of dermatology
2025

Refractory pityriasis rubra pilaris treated with abrocitinib.

Dermatology reports
2025

Psoriasis and Papulosquamous Disorders.

Primary care
2025

Artificial intelligence for the diagnosis of erythematous-squamous dermatological diseases: technological contributions to primary care.

Anais brasileiros de dermatologia
2025

Are Blue Straggler Stars a Hidden Clue? Intriguing Discovery in Pityriasis Rubra Pilaris Under UV-Induced Fluorescence Dermoscopy.

Dermatology practical &amp; conceptual
2025

Efficacy of Bimekizumab in the Management of Refractory Erythrodermic Pityriasis Rubra Pilaris: Clinical Insights.

Dermatology practical &amp; conceptual
2025

Precision, Research, Progress: Updates in the Management of Pityriasis Rubra Pilaris.

Journal of drugs in dermatology : JDD
2025

Case Report: Successful treatment of a novel variant of CARD14-mutated juvenile Pityriasis rubra pilaris with ixekizumab.

Frontiers in medicine
2025

Sorafenib-Induced Pityriasis Rubra Pilaris-like Eruption: A Case Report.

Indian dermatology online journal
2025

Quality of Life Assessment Among Patients with Papulosquamous Disorders in Southern Nigeria.

West African journal of medicine
2025

Treatment of refractory pityriasis rubra pilaris with biologic therapy: a case series.

Clinical and experimental dermatology
2025

Pityriasis rubra pilaris with symmetric polyarthritis and lymphadenopathy.

BMJ case reports
2025

Symmetrical intertriginous drug-related flexural exanthema-like presentation in paediatric patients following bone marrow transplant: think graft-versus-host disease.

Clinical and experimental dermatology
2025

Complete Resolution of Pityriasis Rubra Pilaris With Targeted Treatment: A Case Report.

Cureus
2025

Clinicopathological and management outcome in 70 patients with pityriasis rubra pilaris: A retrospective analysis.

Indian journal of dermatology, venereology and leprology
2025

IL-17C and Pityriasis Rubra Pilaris: Successful Treatment of Erythroderma With Brodalumab.

International journal of dermatology
2025

Pityriasis rubra pilaris following Shingrix vaccine: a report of two cases.

Clinical and experimental dermatology
2025

Classical Juvenile Pityriasis Rubra Pilaris Treated With Secukinumab: Case Report and a Review of Biological Treatments in the Pediatric Population.

Pediatric dermatology
2025

Case Report of Dermatomyositis With Features of the Wong Variant Developing Post-Trastuzumab Therapy for Breast Cancer.

The Australasian journal of dermatology
2025

[Translated article] Facial Discoid Dermatosis. A 13-Case Series.

Actas dermo-sifiliograficas
2025

Efficacy and safety of small molecule drugs in the treatment of pityriasis rubra pilaris-A systematic review.

Frontiers in medicine
2025

Similar Molecular Features in Two Cases of CARD14-Associated Papulosquamous Eruption.

The Australasian journal of dermatology
2025

[Nail changes in inflammatory dermatoses: recognition and treatment].

Dermatologie (Heidelberg, Germany)
2025

Pityriasis Rubra Pilaris Following COVID-19 Infection: A Case of Successful Treatment With Ixekizumab.

Cureus
2025

KLK11-Related Disorder of Cornification Presenting as Inflammatory Skin Disease: A Familial Case Report and Literature Review.

American journal of medical genetics. Part A
2025

Treatment of pityriasis rubra pilaris with systemic and biological agents: A single-center retrospective cohort of 76 patients.

Journal of the American Academy of Dermatology
2025

Targeted dual biologic therapy for erythroderma of unknown etiology guided by high-parameter peripheral blood immunophenotyping.

Scientific reports
2025

A case of pityriasis rubra pilaris secondary to ponatinib.

SAGE open medical case reports
2025

Cytokine Profiling of Erythroderma Biopsies Reveals Types 2 and 17 Immune Activation Status.

Journal of cutaneous pathology
2024

Off-label dermatologic uses of IL-23 inhibitors.

The Journal of dermatological treatment
2024

Atypical adult type pityriasis rubra pilaris.

Indian journal of dermatology, venereology and leprology
2025

Grover's disease-like patterns in early pityriasis rubra pilaris.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2024

Uncommon Presentation of Pityriasis Rubra Pilaris of the Scalp: Clinical, Trichoscopic, and Histopathologic Features and Review of the Literature.

Medicina (Kaunas, Lithuania)
2025

Erythroderma in the elderly.

The Journal of dermatology
2025

Pityriasis rubra pilaris.

Journal of the American Academy of Dermatology
2024

Iatrogenic Hypertriglyceridemia when Treating Pityriasis Rubra Pilaris: An algorithm for Monitoring and Treatment during Retinoid Therapy.

Skinmed
2024

Long-Standing Remission After Tildrakizumab Treatment in a Case of Refractory Type I Pityriasis Rubra Pilaris in a Breast Cancer Patient.

Clinical, cosmetic and investigational dermatology
2024

A young female with rapid progression of erythroderma and palmoplantar keratoderma.

International journal of rheumatic diseases
2024

Revisiting risankizumab: a newer biologic drug in dermatology.

Italian journal of dermatology and venereology
2024

Erythroderma Following Pfizer-BioNTech COVID-19 Vaccination in a Patient With Atopic Dermatitis: A Case Report.

Cureus
2025

Pityriasis rubra pilaris and underlying malignancy.

QJM : monthly journal of the Association of Physicians
2024

Pityriasis rubra pilaris with eosinophilia in a young patient: a case report.

Annals of medicine and surgery (2012)
2024

Wong-Type Dermatomyositis: Literature Review of a Rare Variant.

Journal of clinical rheumatology : practical reports on rheumatic &amp; musculoskeletal diseases
2024

Off-Label Uses of Abrocitinib: Review of Emerging Therapeutic Applications beyond Atopic Dermatitis.

Life (Basel, Switzerland)
2025

Clinical Characteristics and Treatment Patterns of Pityriasis Rubra Pilaris: A Canadian Retrospective Study.

Journal of cutaneous medicine and surgery
2024

Successful Treatment of Refractory Extensive Pityriasis Rubra Pilaris With Risankizumab and Acitretin.

Cutis
2024

Pityriasis Rubra Pilaris in an Atopic Dermatitis Patient: A Case Report.

Cureus
2024

Tofacitinib for Pityriasis Rubra Pilaris: A Case Report.

Clinical, cosmetic and investigational dermatology
2024

Emerging Role of Biologic Drugs Targeting IL-17 and IL-23: Pityriasis Rubra Pilaris.

Life (Basel, Switzerland)
2025

Hyperactivation of the IL-17 Axis and IL-36 Signaling in Card14-Mutant Pityriasis Rubra Pilaris Mouse Model.

The Journal of investigative dermatology
2024

Guselkumab - In Psoriasis and Beyond.

Dermatology practical &amp; conceptual
2024

Severe atypical juvenile pityriasis rubra pilaris diagnosed in adulthood with a dramatic improvement with ustekinumab.

Skin health and disease
2024

A case report of refractory advanced-stage mycosis fungoides: successful treatment and improved patient quality of life with mogamulizumab.

Therapeutic advances in hematology
2024

Successful Treatment of Generalized Pustular Psoriasis Coexisting with Pityriasis Rubra Pilaris with Upadacitinib.

Indian journal of pediatrics
2024

Loss-of-function mutations in Keratin 32 gene disrupt skin immune homeostasis in pityriasis rubra pilaris.

Nature communications
2025

JAAD Game Changers: CARD14-associated papulosquamous eruption: A spectrum including features of psoriasis and pityriasis rubra pilaris.

Journal of the American Academy of Dermatology
2024

Rapid clearance of extensive juvenile pityriasis rubra pilaris with ixekizumab.

Pediatric dermatology
2024

Severe pityriasis rubra pilaris complicated with Kaposi's varicelliform eruption and cutaneous MRSA infection case report.

Heliyon
2024

Pityriasis Rubra Pilaris - a difficult path to optimal treatment. Case report.

Polski merkuriusz lekarski : organ Polskiego Towarzystwa Lekarskiego
2024

Targeting IL-1 controls refractory pityriasis rubra pilaris.

Science advances
2024

Post-infectious pityriasis rubra pilaris in pediatric patients.

Archivos argentinos de pediatria
2024

Role of IL-23 inhibitors including risankizumab and guselkumab in the treatment of pityriasis rubra pilaris.

Archives of dermatological research
2024

Surgical Management of Severe Cicatricial Ectropion Secondary to Pityriasis Rubra Pilaris.

Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]
2024

Erythrodermic Pityriasis Rubra Pilaris Following COVID-19 Vaccination.

Cutis
2024

Tildrakizumab use for recalcitrant pityriasis rubra pilaris.

The Australasian journal of dermatology
2024

Risankizumab for the treatment of pityriasis rubra pilaris postanaplastic large cell lymphoma.

JAAD case reports
2024

Ophthalmic complication of pityriasis rubra pilaris.

BMJ case reports
2024

Successful treatment of pityriasis rubra pilaris with brodalumab after nonresponse to IL-17A inhibition: A role for the innate cytokine IL-17C.

JAAD case reports
2024

Early Gestational Diabetes Mellitus: An Update.

The Journal of the Association of Physicians of India
2024

Inappropriate Use of Steroids in Superficial Dermatophytosis: An Uncommon Case of Erythroderma.

The Journal of the Association of Physicians of India
2024

Rapid improvement of severe pityriasis rubra pilaris upon treatment with bimekizumab.

International journal of dermatology
2024

Pityriasis rubra pilaris after COVID-19 vaccination: successful treatment with ustekinumab.

Anais brasileiros de dermatologia
2024

Two Incidental Sibling Diagnoses of Netherton Syndrome in Separate Visits: A Case Report.

Cureus
2024

Pityriasis following COVID-19 vaccinations: a systematic review.

Dermatology reports
2024

Guselkumab for Pityriasis Rubra Pilaris and Dysregulation of IL-23/IL-17 and NFkB Signaling: A Nonrandomized Trial.

JAMA dermatology
2024

Efficacy and safety of oral abrocitinib monotherapy in pityriasis rubra pilaris.

Journal of the European Academy of Dermatology and Venereology : JEADV
2024

Biologics for Treatment of Pityriasis Rubra Pilaris: A Literature Review.

Journal of cutaneous medicine and surgery
2024

Diseases categorized as autoinflammatory keratinization diseases (AiKDs), and their pathologies and treatments.

Nagoya journal of medical science
2024

Pityriasis Rubra Pilaris: A Multicentric Case Series of 65 Spanish Patients.

Actas dermo-sifiliograficas
2024

Case report: Successful treatment with biologics in a pediatric patient with a severe inflammatory skin disease and novel CARD14 mutation.

Frontiers in medicine
2023

An Institution Based Cross-Sectional Observational Aetiological Study Correlating the Clinico-Pathological Findings in Patients Presenting with Acquired Palmoplantar Keratoderma.

Indian journal of dermatology
2024

Sezary syndrome initially presenting as pityriasis rubra pilaris: Clinicopathologic study of 3 cases.

JAAD case reports
2024

Delayed Presentation of Pityriasis Rubra Pilaris in a Patient on Treatment With Ponatinib.

Cureus
2025

Resolution of Pityriasis rubra pilaris induced ectropion with oral dexamethasone. A case report.

Orbit (Amsterdam, Netherlands)
2023

Key Factors in the Complex and Coordinated Network of Skin Keratinization: Their Significance and Involvement in Common Skin Conditions.

International journal of molecular sciences
2024

Updates on Pityriasis Rubra Pilaris: A Scoping Review.

Journal of cutaneous medicine and surgery
2024

Pityriasis Rubra Pilaris: An Updated Review of Clinical Presentation, Etiopathogenesis, and Treatment Options.

American journal of clinical dermatology
2024

HIV: Inflammatory dermatoses.

Clinics in dermatology
2024

Biologics for inherited disorders of keratinisation: A systematic review.

The Australasian journal of dermatology
2023

Autoinflammatory Keratinization Diseases-The Concept, Pathophysiology, and Clinical Implications.

Clinical reviews in allergy &amp; immunology
2024

Successful treatment of erythrodermic pityriasis rubra pilaris with upadacitinib.

Journal of the European Academy of Dermatology and Venereology : JEADV
2023

Ponatinib Induced Pityriasis Rubra Pilaris: Case Report and Review of Literature.

Indian dermatology online journal
2023

Utility of Dermoscopy in the Diagnosis of Erythroderma: A Cross-Sectional Study.

Indian dermatology online journal
2023

Case report: Area of focus of management of severe pityriasis rubra pilaris by dose optimization of adalimumab biosimilar in combination with acitretin and montelukast.

Frontiers in medicine
2024

Successful treatment of CARD14-negative juvenile Pityriasis rubra pilaris with Ustekinumab.

Journal of the European Academy of Dermatology and Venereology : JEADV
2024

Bimekizumab in refractory pityriasis rubra pilaris.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2024

Methotrexate and acitretin in pityriasis rubra pilaris: A retrospective cohort study.

Journal of the American Academy of Dermatology
2023

Erythrodermic pityriasis rubra pilaris following SARS-CoV-2 vaccination treated with bimekizumab.

JAAD case reports
2023

Eosinophils in Traditionally Noneosinophil-Rich Dermatoses.

The American Journal of dermatopathology
2024

Rapid response of erythrodermic pityriasis rubra pilaris to tildrakizumab.

Clinical and experimental dermatology
2023

Wong-type dermatomyositis with interstitial lung disease and anti-SRP and -PM/Scl antibodies treated with intravenous immunoglobulin.

JAAD case reports
2023

Corrigendum to "Successful treatment of a child's pityriasis rubra pilaris (PRP)with ustekinumab and acitretin"1.

Pediatric dermatology
2023

Acute Exfoliative Dermatitis/Erythroderma Secondary to Gliclazide.

Cureus
2024

Patients with keratinization disorders due to ABCA12 variants showing pityriasis rubra pilaris phenotypes.

The Journal of dermatology
2024

Patient-reported cutaneous signs and symptoms of adult pityriasis rubra pilaris and correlation with quality of life and clinician-reported severity: A cross-sectional study.

Journal of the American Academy of Dermatology
2023

A Rare Post-infectious Rash: Pityriasis Rubra Pilaris After COVID-19 Infection.

Cureus
2023

Successful Treatment of Pityriasis Rubra Pilaris with Risankizumab in Children.

Dermatology and therapy
2023

Nail Whispers Revealing Dermatological and Systemic Secrets: An Analysis of Nail Disorders Associated With Diverse Dermatological and Systemic Conditions.

Cureus
2023

A case of paraneoplastic pityriasis rubra pilaris.

JAAD case reports
2023

Biologic treatment options for pityriasis rubra pilaris: An evidence-based systematic review.

Journal of the American Academy of Dermatology
2023

The individual Pityriasis Rubra Pilaris area and severity index (iPRPASI): validity, reliability, and responsiveness of a novel patient-reported severity tool.

Archives of dermatological research
2023

The Effectiveness of Anti-Interleukin-17A Treatment for Pityriasis Rubra Pilaris: A Systematic Review.

Cureus
2023

Pityriasis rubra pilaris with simultaneous emergence of human herpesvirus-6 reactivation in a patient with drug-induced hypersensitivity syndrome.

The Journal of dermatology
2023

Pityriasis rubra pilaris induced by topical use of imiquimod 5.

Anais brasileiros de dermatologia
2023

Kaposi's Varicelliform Eruption After Treatment With Ixekizumab in a Patient With Pityriasis Rubra Pilaris.

Cureus
2023

A child with CARD14-associated papulosquamous eruption (CAPE) successfully treated with ustekinumab.

Pediatric dermatology
2023

A case of follicular psoriasis mimicking pityriasis rubra pilaris: a diagnostic dilemma.

International journal of dermatology
2023

A case of pityriasis rubra pilaris treated with tofacitinib after failure with acitretin and ixekizumab.

The Australasian journal of dermatology
2023

Juvenile Pityriasis Rubra Pilaris.

Dermatology practical &amp; conceptual
2023

Misdiagnosed Pityriasis Rubra Pilaris Successfully Managed With Isotretinoin: A Case Series.

Cureus
2023

Refractory pityriasis rubra pilaris treated with upadacitinib.

JAAD case reports
2023

Pityriasis rubra pilaris following COVID-19 vaccination successfully treated with ixekizumab.

JAAD case reports
2023

Pityriasis rubra pilaris partially responsive to treatment with upadacitinib: A case report.

SAGE open medical case reports
2023

Clinical characteristics of patients with pityriasis rubra pilaris following SARS-CoV-2 vaccination.

Journal of the European Academy of Dermatology and Venereology : JEADV
2022

Pityriasis Rubra Pilaris Significantly Improved after Treatment for Chronic Focal Infection.

Indian journal of dermatology
2022

Juvenile Pityriasis Rubra Pilaris in a 4-year-old Child Treated Successfully with Secukinumab.

Indian journal of dermatology
2023

A novel mutation in a CARD14-associated papulosquamous eruption.

Pediatric dermatology
2023

Pityriasis rubra pilaris type IV-associated arthritis treated with adalimumab.

International journal of rheumatic diseases
2023

Rethinking pityriasis rubra pilaris as a paraneoplastic syndrome: Two cases of pityriasis rubra pilaris with concomitant underlying malignancy.

JAAD case reports
2023

A case of bullous pemphigoid developing after ixekizumab therapy for pityriasis rubra pilaris.

The Journal of dermatology
2023

Pityriasis Rubra Pilaris After Moderna COVID-19 Vaccination: A Case Report and Literature Review.

The American Journal of dermatopathology
2022

Cutaneous and Developmental Effects of CARD14 Overexpression in Zebrafish.

Biomedicines
2023

Treatment Options for Juvenile Pityriasis Rubra Pilaris.

Paediatric drugs
2023

Pityriasis rubra pilaris following inactivated SARS-CoV-2 vaccine (CoronaVac).

Journal of the European Academy of Dermatology and Venereology : JEADV
2023

Ponatinib-Associated Cutaneous Eruptions-A Case Series and Review of Clinicopathologic Findings.

The American Journal of dermatopathology
2022

Physiotherapy Management of an Adolescent With Pityriasis Rubra Pilaris Along With Rickets.

Cureus
2022

Pityriasis rubra pilaris with rickets: a rare clinical image.

The Pan African medical journal
2022

Atypical Juvenile Pityriasis Rubra Pilaris: A Case Report of Early Onset With Late Diagnosis.

Cureus
2022

Pityriasis rubra pilaris heralding diagnosis of urothelial carcinoma: a case report.

Dermatology online journal
2022

Successful treatment with cyclosporine and guselkumab for pityriasis rubra pilaris.

Clinical case reports
2022

Successful treatment with ustekinumab in CARD14-associated papulosquamous eruption in a Brazilian child.

Dermatologic therapy
2022

Off-label uses of ustekinumab.

Dermatologic therapy
2022

Pityriasis rubra pilaris (type I) following administration of the BNT162b2 mRNA COVID-19 vaccine: Successful treatment with ustekinumab and acitretin.

Dermatologic therapy
2023

Pityriasis Rubra Pilaris Transcriptomics Implicate T Helper 17 Signaling and Correlate with Response to Ixekizumab, with Distinct Gene Expression Profiles in Nonresponders.

The Journal of investigative dermatology
2022

A Case of Ichthyosis Vulgaris and the Use of 70% Glycolic Acid Chemical Peels for Management.

Cureus
2022

Efficacy of ustekinumab and guselkumab in treatment refractory pityriasis rubra pilaris: A case series.

The Australasian journal of dermatology
2022

Pityriasis rubra pilaris rapidly cleared with ixekizumab in an HIV-positive patient.

JAAD case reports
2022

Pityriasis rubra pilaris following booster dose of mRNA (Pfizer-BioNTech) COVID-19 vaccine.

Dermatologic therapy
2022

Anti-interleukin-17A for pityriasis rubra pilaris: catching the psoriasis biologic wave.

The British journal of dermatology
2023

COVID-19 induced pityriasis rubra pilaris: A superantigenic disease?

Journal of the European Academy of Dermatology and Venereology : JEADV
2022

Early Presentation of Pityriasis Rubra Pilaris Mimicking Tinea Corporis: Diagnostic Challenges of a Rare Skin Condition.

The American journal of case reports
2022

Pityriasis rubra pilaris with erythema gyratum repens-like eruption and resolution with ustekinumab.

Clinical and experimental dermatology
2023

Successful treatment of pityriasis rubra pilaris with risankizumab, a IL-23/p19 antagonist.

Journal of the European Academy of Dermatology and Venereology : JEADV
2022

Ixekizumab successfully treated severe pityriasis rubra pilaris after COVID-19 vaccination.

Skin health and disease
2022

Clinical course, diagnostic patterns, and treatment outcomes in patients with pityriasis rubra pilaris.

Journal of the American Academy of Dermatology
2022

Successful treatment of severe pityriasis rubra pilaris with secukinumab in a 3-year-old boy.

Clinical and experimental dermatology
2022

Patient and disease characteristics associated with psychiatric symptoms and impaired quality of life in pityriasis rubra pilaris.

The British journal of dermatology
2022

Ustekinumab-Induced Fatal Acute Heart Failure in a Young Female: A Case Report.

Methodist DeBakey cardiovascular journal
2022

Successful treatment of a child's pityriasis rubra pilaris (PRP) with ustekinumab and acitretin.

Pediatric dermatology
2022

Paraneoplastic Pityriasis Rubra Pilaris Preceding Leukemia.

Advances in skin &amp; wound care
2022

Secukinumab for the treatment of adult-onset pityriasis rubra pilaris: a single-arm clinical trial with transcriptomic analysis.

The British journal of dermatology
2022

Beyond plaque psoriasis - pathogenesis and treatment of other psoriasis phenotypes.

Current opinion in rheumatology
2021

Cutaneous sarcoidosis due to immune-checkpoint inhibition and exacerbated by a novel BRAF dimerization inhibitor.

Skin health and disease
2022

Delayed-onset psoriasiform eruption secondary to a phosphoinositide 3-kinase inhibitor: A case report and literature review.

JAAD case reports
2022

Erythrodermic pityriasis rubra pilaris after SARS-CoV-2 vaccination with concomitant COVID-19 infection.

Journal of the European Academy of Dermatology and Venereology : JEADV
2022

A case of pityriasis rubra pilaris following AstraZeneca COVID-19 vaccine.

JAAD case reports
2022

Pityriasis rubra pilaris potentially triggered by messenger RNA-1273 COVID vaccine.

JAAD case reports
2022

A case of classic adult pityriasis rubra pilaris successfully treated with a combination of acitretin and ustekinumab: A case report.

SAGE open medical case reports
2022

Acute postinfectious type III pityriasis rubra pilaris as a cutaneous manifestation in COVID-19: Decoding a possible trigger!!

Journal of cosmetic dermatology
2022

Eine neue Form der juvenilen Pityriasis rubra pilaris mit möglicher Beteiligung bakterieller Superantigene.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2022

Pityriasis rubra pilaris in association with inactivated SARS-CoV-2 vaccine (CoronaVac).

Dermatologic therapy
2022

[Pityriasis rubra pilaris after COVID-19 vaccination: causal relationship or coincidence?].

Dermatologie (Heidelberg, Germany)
2022

Pityriasis rubra pilaris (type I) following ChAdOx1 COVID-19 vaccine: A report of two cases with successful treatment with oral isotretinoin.

Journal of the European Academy of Dermatology and Venereology : JEADV
2022

Dermoscopy of Gottron's papules and other inflammatory dermatoses involving the dorsa of the hands.

Journal of the European Academy of Dermatology and Venereology : JEADV
2022

Clinical response of CARD14-associated papulosquamous eruption to an anti-interleukin-17A antibody.

The British journal of dermatology
2022

Keratosis pilaris rubra successfully treated with topical sirolimus: Report of a case and review of the literature.

Pediatric dermatology
Ver todos os 800 no EuropePMC

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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Successful Treatment of Pityriasis Rubra Pilaris With Deucravacitinib.
    JAMA dermatology· 2026· PMID 41563745mais citado
  2. CARD14-Associated Papulosquamous Eruption (CAPE): An Updated Review of Pathogenesis and Treatment.
    International journal of dermatology· 2026· PMID 41845528mais citado
  3. Case Report: Successful treatment of pityriasis rubra pilaris with deucravacitinib.
    Frontiers in immunology· 2026· PMID 41716399mais citado
  4. Vitamins and the skin: Vitamin A and retinoids in dermatology.
    Clinics in dermatology· 2026· PMID 41692081mais citado
  5. Pityriasis rubra pilaris triggered by vaccination.
    Italian journal of dermatology and venereology· 2026· PMID 41379524mais citado
  6. The use of upadacitinib for refractory pityriasis rubra pilaris: A case report.
    JAAD Case Rep· 2026· PMID 41953854recente
  7. Successful Treatment of Two Rare Pediatric Keratinization Disorders with Secukinumab: Epidermolytic Ichthyosis and PRP-GPP Overlap.
    Psoriasis (Auckl)· 2026· PMID 41940178recente
  8. Comparing Clinical vs Histopathological Features in Diagnosing Erythemato-Squamous Diseases.
    J Clin Investig Dermatol· 2026· PMID 41924630recente
  9. Successful Treatment of Pityriasis Rubra Pilaris with Upadacitinib: Report of Two Cases.
    Acta Derm Venereol· 2026· PMID 41885793recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:2897(Orphanet)
  2. OMIM OMIM:173200(OMIM)
  3. MONDO:0008251(MONDO)
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. Artigo Wikipedia(Wikipedia)
  7. Q766856(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Pytiriasis rubra pilosa
Compêndio · Raras BR

Pytiriasis rubra pilosa

ORPHA:2897 · MONDO:0008251
Prevalência
1-9 / 1 000 000
Casos
48 casos conhecidos
Herança
Autosomal dominant, Not applicable
CID-10
L44.0 · Pitiríase rubra pilar
CID-11
Ensaios
2 ativos
Início
All ages
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0032027
EuropePMC
Wikidata
Wikipedia
Papers 10a
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