Raras
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Acrodermatite contínua de Hallopeau
ORPHA:163931CID-10 · L40.2CID-11 · EA90.41OMIM 614204DOENÇA RARA

Psoríase que tem como causa uma mutação no gene IL36RN.

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Introdução

O que você precisa saber de cara

📋

Psoríase que tem como causa uma mutação no gene IL36RN.

Publicações científicas
229 artigos
Último publicado: 2026
Medicamentos
6 registrados
USTEKINUMAB, GUSELKUMAB, IMSIDOLIMAB

Tem tratamento?

6 medicamentos registrados
Ver detalhes, fases e interações →
USTEKINUMABGUSELKUMABIMSIDOLIMABADALIMUMABSPESOLIMABCERTOLIZUMAB PEGOL

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 100 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
6.0
Worldwide
Início
Adult
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: L40.2
Você se identifica com essa condição?
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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

Partes do corpo afetadas

🧬
Pele e cabelo
2 sintomas
🩸
Sangue
2 sintomas
🦴
Ossos e articulações
1 sintomas
💪
Músculos
1 sintomas

+ 9 sintomas em outras categorias

Características mais comuns

100%prev.
Acantose epidérmica
Frequência: 8/8
100%prev.
Paraqueratose
Frequência: 8/8
100%prev.
Concentração elevada de proteína C-reativa circulante
Frequência: 30/30
100%prev.
Dermatite psoriasiforme
Frequência: 16/16
100%prev.
Neutrofilia
Frequência: 14/14
100%prev.
Leucocitose
Frequência: 16/16
15sintomas
Muito frequente (6)
Ocasional (6)
Sem dados (3)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 15 características clínicas mais associadas, ordenadas por frequência.

Acantose epidérmicaEpidermal acanthosis
Frequência: 8/8100%
ParaqueratoseParakeratosis
Frequência: 8/8100%
Concentração elevada de proteína C-reativa circulanteElevated circulating C-reactive protein concentration
Frequência: 30/30100%
Dermatite psoriasiformePsoriasiform dermatitis
Frequência: 16/16100%
NeutrofiliaNeutrophilia
Frequência: 14/14100%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico229PubMed
Últimos 10 anos160publicações
Pico202523 papers
Linha do tempo
2026Hoje · 2026🧪 2013Primeiro ensaio clínico📈 2025Ano 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

2 genes identificados com associação a esta condição. Padrão de herança: Autosomal recessive.

AP1S3AP-1 complex subunit sigma-3Disease-causing germline mutation(s) inTolerante
FUNÇÃO

Subunit of clathrin-associated adaptor protein complex 1 that plays a role in protein sorting in the late-Golgi/trans-Golgi network (TGN) and/or endosomes. The AP complexes mediate both the recruitment of clathrin to membranes and the recognition of sorting signals within the cytosolic tails of transmembrane cargo molecules. Involved in TLR3 trafficking (PubMed:24791904)

LOCALIZAÇÃO

Golgi apparatusCytoplasmic vesicle membraneMembrane, clathrin-coated pit

VIAS BIOLÓGICAS (2)
MHC class II antigen presentationLysosome Vesicle Biogenesis
MECANISMO DE DOENÇA

Psoriasis 15, pustular

A form of pustular psoriasis, a life-threatening disease defined by repeated flares of sudden onset consisting of diffuse erythematous skin eruption characterized by rapid coverage with pustules, high-grade fever, asthenia, marked leukocytosis, and elevated serum levels of C-reactive protein.

OUTRAS DOENÇAS (4)
psoriasis 14, pustularpustulosis palmaris et plantarisgeneralized pustular psoriasispsoriasis 15, pustular, susceptibility to
HGNC:18971UniProt:Q96PC3
IL36RNInterleukin-36 receptor antagonist proteinDisease-causing germline mutation(s) inTolerante
FUNÇÃO

Inhibits the activity of interleukin-36 (IL36A,IL36B and IL36G) by binding to receptor IL1RL2 and preventing its association with the coreceptor IL1RAP for signaling. Part of the IL-36 signaling system that is thought to be present in epithelial barriers and to take part in local inflammatory response; similar to the IL-1 system with which it shares the coreceptor. Proposed to play a role in skin inflammation. May be involved in the innate immune response to fungal pathogens, such as Aspergillus

LOCALIZAÇÃO

CytoplasmSecreted

VIAS BIOLÓGICAS (1)
Interleukin-36 pathway
MECANISMO DE DOENÇA

Psoriasis 14, pustular

A life-threatening disease defined by repeated flares of sudden onset consisting of diffuse erythematous skin eruption characterized by rapid coverage with pustules, high-grade fever, asthenia, marked leukocytosis, and elevated serum levels of C-reactive protein.

VIAS REACTOME (1)
EXPRESSÃO TECIDUAL(Tecido-específico)
Skin Sun Exposed Lower leg
82.0 TPM
Skin Not Sun Exposed Suprapubic
49.1 TPM
Esôfago - Mucosa
21.0 TPM
Vagina
3.8 TPM
Testículo
0.2 TPM
OUTRAS DOENÇAS (3)
psoriasis 14, pustulargeneralized pustular psoriasispustulosis palmaris et plantaris
HGNC:15561UniProt:Q9UBH0

Medicamentos e terapias

USTEKINUMABPhase 3

Mecanismo: Interleukin-23 inhibitor

GUSELKUMABPhase 3

Mecanismo: Interleukin-23 inhibitor

IMSIDOLIMABPhase 3

Mecanismo: IL36 receptor antagonist

ADALIMUMABPhase 3

Mecanismo: TNF-alpha inhibitor

SPESOLIMABPhase 3

Mecanismo: IL36 receptor antagonist

CERTOLIZUMAB PEGOLPhase 2

Mecanismo: TNF-alpha inhibitor

Ver mais no OpenTargets

Variantes genéticas (ClinVar)

67 variantes patogênicas registradas no ClinVar.

🧬 IL36RN: NM_012275.3(IL36RN):c.41C>A (p.Ser14Ter) ()
🧬 IL36RN: NM_012275.3(IL36RN):c.205_212del (p.Ser69fs) ()
🧬 IL36RN: NM_012275.3(IL36RN):c.273del (p.Ala92fs) ()
🧬 IL36RN: NM_012275.3(IL36RN):c.16del (p.Ala6fs) ()
🧬 IL36RN: GRCh37/hg19 2q12.2-21.2(chr2:106755586-134302739)x1 ()
Ver todas no ClinVar

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.
3Fase 35
2Fase 22
Medicamentos catalogadosEnsaios clínicos· 6 medicamentos · 1 ensaio
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Acrodermatite contínua de Hallopeau

🗺️

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

Pesquisa e ensaios clínicos

3 ensaios clínicos encontrados.

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

Timeline de publicações
160 papers (10 anos)
#1

Case Report: Periungual Xeligekimab injection for refractory Acrodermatitis continua of Hallopeau.

Frontiers in immunology2026

This case report presents the novel and successful use of periungual Xeligekimab injections for managing refractory Acrodermatitis Continua of Hallopeau (ACH), representing the first documented application of this localized administration route for the IL-17A inhibitor. It contributes to the scientific literature by demonstrating a promising alternative therapeutic strategy for treatment-resistant ACH, highlighting the potential for enhanced local efficacy and minimized systemic exposure. The patient was a 36-year-old woman with a two-decade history of ACH affecting her digits, characterized by persistent periungual and subungual erythema, sterile pustules, significant nail plate dystrophy with thickening and fragmentation, and associated digital swelling, tenderness, and restricted motion. Prior therapies, including topical corticosteroids, phototherapy, and systemic tofacitinib, had proven ineffective. Diagnosis was confirmed clinically and supported by MRI, which revealed active bone marrow edema in the distal phalanges. The therapeutic intervention involved initial periungual injections of a diluted Xeligekimab formulation, which led to partial improvement. This was followed by a series of injections using undiluted Xeligekimab (100 mg/mL). This escalation resulted in marked clinical resolution: complete clearance of pustules and inflammation, healthy nail regrowth, and resolution of digital swelling and pain. Concurrently, the Dermatology Life Quality Index and pain scores dramatically improved, and follow-up MRI showed substantial resolution of the underlying bone marrow edema, confirming the treatment's efficacy on both cutaneous and deep osseous inflammation.

#2

Dual IL-17A/F Blockade for Acrodermatitis Continua of Hallopeau: A Clinical Response to Bimekizumab.

Journal of drugs in dermatology : JDD2026 Feb 01

Acrodermatitis continua of Hallopeau (ACH) is a rare, localized variant of pustular psoriasis that primarily affects the distal digits and nail apparatus, often presenting with recurrent pustules, nail dystrophy, and significant functional impairment. Due to its rarity and chronic relapsing course, ACH is notoriously difficult to treat, and standardized treatment guidelines are lacking. We present the case of a 67-year-old male with ACH who failed multiple therapies, including corticosteroids, topical tapinarof, and oral deucravacitinib, before achieving rapid and sustained improvement with bimekizumab, a monoclonal antibody targeting interleukin-17A and IL-17F. Within one month of initiating bimekizumab, the patient experienced marked clinical improvement in both skin lesions and joint pain, with continued progress allowing him to return to work. This case highlights the potential utility of dual IL-17A/F inhibition in neutrophil-dominant pustular conditions such as ACH. As more case reports document favorable outcomes, bimekizumab may emerge as a valuable treatment option for patients with refractory ACH, offering targeted cytokine blockade in a condition with few effective therapies. &nbsp.

#3

Clinical Features and Long-Term Outcomes in 109 Pediatric Generalized Pustular Psoriasis.

Dermatology (Basel, Switzerland)2026

Pediatric-onset generalized pustular psoriasis (PGPP) is a rare inflammatory disorder characterized by recurrent flares and challenging management. This study aimed to review the clinical features, flare patterns, and treatment options. We analyzed PGPP patients diagnosed at Beijing Children's Hospital from 2006 to 2023 who had complete medical record. Data on clinical characteristics, flare numbers, and flare duration were collected through medical record and telephone survey. The flare frequency was calculated by dividing the flare numbers by the years in each age range. Flare duration was categorized as ≤4 weeks or >4 weeks. In addition, we investigated the factors associated with flare frequency in PGPP patients. A total of 109 patients with PGPP were included in the study, with a follow-up duration ranging from 1.90 to 22.80 years. The median age of onset was 6.00 years, with a higher prevalence in males (73, 66.97%). Most patients (55, 50.46%) experienced their first flare between the ages of 7 and 12 years. A total of 45 patients (41.28%) had history of psoriasis vulgaris, 16 (14.68%) had history of acrodermatitis continua of Hallopeau, and 4 (3.67%) had history of psoriatic arthritis. Respiratory infection was the most common trigger, affecting 35 patients (32.11% of cases). The annual flare frequency was 0.68. The frequency and duration of flares showed a decreasing trend with increasing age. Earlier onset age is associated with a higher flare frequency. Before 2020, conventional therapies were most commonly used, with acitretin used in 67 patients (61.47%). Following 2020, secukinumab became the most often used medication (44 patients, 40.37%). PGPP exhibits a trend toward flare decrease with age, yet recurrence remains common. Earlier onset age may be associated with higher flare frequency. At present, biologic agents are the main treatment. These findings provide new insights into PGPP.

#4

Response to Han et al., "Comment on Iorizzo's acrodermatitis continua of Hallopeau-clinical review and proposed management algorithm".

Journal of the American Academy of Dermatology2026 Apr
#5

Comment on Iorizzo et al, "Acrodermatitis continua of Hallopeau-clinical review and proposed management algorithm".

Journal of the American Academy of Dermatology2026 Apr

Publicações recentes

Ver todas no PubMed

📚 EuropePMC176 artigos no totalmostrando 156

2026

Case Report: Periungual Xeligekimab injection for refractory Acrodermatitis continua of Hallopeau.

Frontiers in immunology
2026

Dual IL-17A/F Blockade for Acrodermatitis Continua of Hallopeau: A Clinical Response to Bimekizumab.

Journal of drugs in dermatology : JDD
2025

Clinical phenotype of 504 pustular psoriasis patients from Europe and Egypt: a multi-center observational study.

Clinical and experimental dermatology
2025

Long-term control of refractory acrodermatitis continua of Hallopeau with subcutaneous spesolimab: A case report.

JAAD case reports
2026

Clinical Features and Long-Term Outcomes in 109 Pediatric Generalized Pustular Psoriasis.

Dermatology (Basel, Switzerland)
2025

Rapid Resolution of Pediatric Acrodermatitis Continua of Hallopeau With Spesolimab.

Pediatrics
2025

Successful treatment of severe acrodermatitis continua of Hallopeau with guselkumab.

Dermatology online journal
2025

Vunakizumab for Treatment of Acrodermatitis Continua of Hallopeau: A Case Report and Literature Review.

Psoriasis (Auckland, N.Z.)
2025

Acrodermatitis continua of Hallopeau: a review and update on biological and small molecule targeted immunomodulatory therapies.

Frontiers in immunology
2025

Refractory Acrodermatitis Continua of Hallopeau in a Pediatric Patient: Unveiling Underlying Mixed Connective Tissue Disorder.

The Journal of clinical and aesthetic dermatology
2025

Acrodermatitis continua of Hallopeau-clinical review and proposed management algorithm.

Journal of the American Academy of Dermatology
2025

Spesolimab in generalized pustular psoriasis complicated by acrodermatitis continua of Hallopeau: a case report and mechanistic insights.

Frontiers in immunology
2025

Apremilast Coadministered with Secukinumab for Effective Treatment of Acrodermatitis Continua of Hallopeau: A Case Report.

Clinical, cosmetic and investigational dermatology
2025

Epidemiological, Genetic, Clinical, and Treatment Differences of Generalized Pustular Psoriasis and Acrodermatitis Continua of Hallopeau Across Ethnicities: A Systematic Review.

American journal of clinical dermatology
2025

Acrodermatitis Continua of Hallopeau and Generalised Pustular Psoriasis: Case Reports of Two Different Manifestations of IL36RN Mutation in Siblings.

Psoriasis (Auckland, N.Z.)
2025

Acrodermatitis continua of Hallopeau successfully treated with spesolimab.

Annales de dermatologie et de venereologie
2025

Expanding the therapeutic horizons of spesolimab: a review of off-label applications for inflammatory skin diseases.

The Journal of dermatological treatment
2024

Case report: Successful treatment with spesolimab of acrodermatitis continua of Hallopeau in an older patient without IL36RN mutations.

Frontiers in immunology
2025

Successful treatment of severe acrodermatitis continua of hallopeau with Bimekizumab: A case report.

SAGE open medical case reports
2025

A rare severe acrodermatitis continua of Hallopeau treated with spesolimab.

The Journal of dermatology
2025

Spesolimab in refractory paediatric acrodermatitis continua of Hallopeau, a case series.

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

Acrodermatitis Continua of Hallopeau Successfully Treated With Bimekizumab.

Dermatology practical &amp; conceptual
2024

Acrodermatitis continua of hallopeau: aggravating factors and treatment outcomes of 96 patients.

The Journal of dermatological treatment
2024

Is tofacitinib monotherapy also effective for the treatment of Acrodermatitis continua of Hallopeau?

The Australasian journal of dermatology
2024

Squamous cell carcinoma developing from acrodermatitis continua of Hallopeau in a patient with deficiency of interleukin-36-receptor antagonist.

Italian journal of dermatology and venereology
2025

Refractory acrodermatitis continua of Hallopeau successfully treated by baricitinib: A case report.

The Journal of dermatology
2025

Rapid improvement in refractory acrodermatitis continua of Hallopeau with spesolimab injection.

Clinical and experimental dermatology
2024

Acrodermatitis Continua of Hallopeau Evolving into Psoriatic Monoarthritis: Successfully Treated with Apremilast.

Skinmed
2025

Successful treatment of acrodermatitis continua of Hallopeau coexisting with flexural psoriasis with upadacitinib: a case report.

Clinical and experimental dermatology
2024

Long-term treatment with risankizumab of palmoplantar pustulosis and acrodermatitis continua of hallopeau.

Italian journal of dermatology and venereology
2025

Effective management of acrodermatitis continua of Hallopeau with guselkumab in a Wiskott-Aldrich syndrome patient.

International journal of dermatology
2024

Plaque psoriasis, generalized pustular psoriasis, palmoplantar pustulosis, and acrodermatitis continua of Hallopeau successfully treated with bimekizumab: a promising therapeutic approach.

European journal of dermatology : EJD
2024

Targeted therapy outcomes in acrodermatitis continua of Hallopeau: A systematic review.

Journal of biomedical research
2025

Reflectance confocal microscopy and dermoscopic features of acrodermatitis continua of Hallopeau.

International journal of dermatology
2024

Acrodermatitis continua of Hallopeau upon PD-L1 therapy for metastatic urothelial carcinoma.

European journal of dermatology : EJD
2024

Generalized acrodermatitis continua of Hallopeau with an IL36RN variant successfully treated with bimekizumab.

The Journal of dermatology
2024

Genetic analysis of different subtypes of aseptic pustulosis in the Chinese population.

Clinical and experimental dermatology
2024

A retrospective analysis of 65 patients with acrodermatitis continua of Hallopeau.

Experimental dermatology
2024

Acquired diffuse palmoplantar erythema with keratoderma in Chinese patients with pustular psoriasis: A predictor for IL36 receptor antagonist c.115+6T>C mutation?

Experimental dermatology
2024

Successful treatment of acrodermatitis continua of Hallopeau coexisting with generalized pustular psoriasis with spesolimab: a case report.

Frontiers in immunology
2024

Spesolimab Response in a Girl With Acrodermatitis Continua of Hallopeau.

JAMA dermatology
2024

Successful treatment of acrodermatitis continua of Hallopeau by TYK2 inhibitor with every other day.

The Journal of dermatological treatment
2023

Low grade of satisfaction related to the use of current systemic therapies among pustular psoriasis patients: a therapeutic unmet need to be fulfilled.

Frontiers in medicine
2024

Local injection of micro-dose guselkumab for acrodermatitis continua of Hallopeau after failure of systemic ixekizumab treatment.

International journal of dermatology
2023

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

Clinical reviews in allergy &amp; immunology
2024

Treatment of Severe Palmoplantar Pustular Psoriasis With Bimekizumab.

JAMA dermatology
2023

Paediatric acrodermatitis continua of Hallopeau: perspectives of a patient and their mother.

Clinical and experimental dermatology
2023

Anti-Interleukin-17s for successful management of pustular psoriasis.

Cytokine
2023

Acrodermatitis continua of Hallopeau with insufficient response to ixekizumab and infliximab, successfully treated with a combination of ustekinumab and tofacitinib.

European journal of dermatology : EJD
2023

Acrodermatitis continua of Hallopeau in an elderly patient successfully and rapidly treated with risankizumab: a case report.

The Journal of dermatological treatment
2023

Successful treatment of acrodermatitis continua of Hallopeau with tildrakizumab: A case report.

SAGE open medical case reports
2023

Pustular psoriasis: A distinct aetiopathogenic and clinical entity.

Indian journal of dermatology, venereology and leprology
2023

Ixekizumab for the Treatment of Acrodermatitis Continua of Hallopeau and Inverse Psoriasis.

Indian journal of dermatology
2023

Biologics for the Treatment of Severe Acrodermatitis Continua of Hallopeau: Report of Two Cases Successfully Treated with Ixekizumab and Ustekinumab.

Dermatology practical &amp; conceptual
2023

Adalimumab treatment for acrodermatitis continua of Hallopeau: three cases refractory to IL-17 inhibition.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2023

Dermoscopy of Acrodermatitis Continua of Hallopeau.

Indian dermatology online journal
2023

Acrodermatitis continua of Hallopeau and generalised pustular psoriasis: Should they be the same or different entities?

Experimental dermatology
2023

Acrodermatitis Continua of Hallopeau Successfully Treated with Ustekinumab: A Case Report and Literature Review.

Clinical, cosmetic and investigational dermatology
2023

Acrodermatitis continua of Hallopeau successfully treated with bimekizumab: A case report.

SAGE open medical case reports
2023

Squamous cell carcinoma mimicking acrodermatitis continua suppurativa in a patient with generalized pustular psoriasis.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2023

Deficiency of interleukin-36 receptor antagonist (DITRA): An analysis of 58 Chinese patients in a tertiary hospital in Taiwan.

Experimental dermatology
2022

Rapid and sustained response to apremilast in a patient with long-standing acrodermatitis continua of Hallopeau.

Dermatology online journal
2023

Acrodermatitis Continua of Hallopeau: A Diagnostic Challenge.

Indian dermatology online journal
2023

Different Clinical Features of Pediatric Generalized Pustular Psoriasis in Patients with or without IL36RN Variants.

Dermatology (Basel, Switzerland)
2022

Acute Generalized Pustular Psoriasis Developed Resistance to Adalimumab Was Successfully Treated with Narrowband Ultraviolet B and Acitretin: A Case Report.

Clinical, cosmetic and investigational dermatology
2022

A rare case of Acrodermatitis continua of Hallopeau successfully treated with topical calcipotriol/betamethasone dipropionate ointment associated with Jaccaud's arthropathy: A case report.

SAGE open medical case reports
2023

Epidemiological survey of patients with pustular psoriasis in the Japanese Society for Psoriasis Research from 2017 to 2020.

The Journal of dermatology
2022

Targeting IL-17A for the treatment of pustular psoriasis: a comprehensive review.

Expert opinion on biological therapy
2022

Clinical Characteristics of Patients with Pustular Psoriasis: A Single-Center Retrospective Observational Study.

Vaccines
2022

Line-field confocal optical coherence tomography: a new tool for non-invasive differential diagnosis of pustular skin disorders.

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

A case of acrodermatitis continua of Hallopeau successfully treated with guselkumab.

Dermatologic therapy
2022

Certolizumab Pegol: A New Therapeutic Approach for Acrodermatitis Continua of Hallopeau.

Skin appendage disorders
2022

Acrodermatitis continua of Hallopeau: historical perspectives and modern management.

Italian journal of dermatology and venereology
2022

Palmoplantar pustulosis and acrodermatitis continua of Hallopeau: demographic and clinical comparative study in a large multicentre cohort.

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

Acrodermatitis continua of Hallopeau: Is apremilast an efficacious treatment option?

Dermatologic therapy
2023

A case of acrodermatitis continua of Hallopeau evolving into generalized pustular psoriasis successfully treated with guselkumab.

International journal of dermatology
2022

Acrodermatitis continua of Hallopeau successfully treated with ixekizumab: A case report.

Dermatologic therapy
2021

Clinico-Pathological and Dermoscopic Features in Acrodermatitis Continua of Hallopeau.

Indian journal of dermatology
2022

Pustular psoriasis as an autoinflammatory keratinization disease (AiKD): Genetic predisposing factors and promising therapeutic targets.

Journal of dermatological science
2021

Pustular Psoriasis: From Pathophysiology to Treatment.

Biomedicines
2021

Treatment and molecular profiling of acrodermatitis continua of Hallopeau during pregnancy using targeted therapy.

JAAD case reports
2021

Pustular Psoriasis: A Narrative Review of Recent Developments in Pathophysiology and Therapeutic Options.

Dermatology and therapy
2021

Successful therapy of acrodermatitis continua of Hallopeau with IL-23 blockers -two new cases.

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

Treating paediatric acrodermatitis continua of Hallopeau with adalimumab: a case series.

Clinical and experimental dermatology
2021

Epidemiology and clinical features of pustular psoriasis: A 15-year retrospective cohort.

The Journal of dermatology
2021

COVID-19 and exacerbation of dermatological diseases: A review of the available literature.

Dermatologic therapy
2021

Clinical background of patients with psoriasiform skin lesions due to tumor necrosis factor antagonist administration at a single center.

The Journal of dermatology
2021

Successful Treatment of Acrodermatitis Continua of Hallopeau with an Anti-IL-17A Agent.

Indian journal of dermatology
2021

Pustular Psoriasis and Associated Musculoskeletal Disorders.

The Journal of rheumatology. Supplement
2021

Acrodermatitis Continua of Hallopeau Evolving into Generalized Pustular Psoriasis Following COVID-19: A Case Report of a Successful Treatment with Infliximab in Combination with Acitretin.

Biologics : targets &amp; therapy
2021

Inhibition of Progression of Acrodermatitis Continua of Hallopeau With Baricitinib.

JAMA dermatology
2021

Squamous cell carcinoma arising on acrodermatitis continua of Hallopeau: clinical and noninvasive skin imaging features.

International journal of dermatology
2021

A brief guide to pustular psoriasis for primary care providers.

Postgraduate medicine
2020

[Acrodermatitis continua of Hallopeau and psoriatic arthritis: A case report].

Medwave
2020

Brodalumab in the treatment of recalcitrant acrodermatitis continua of Hallopeau.

Dermatologic therapy
2020

Three cases of IL36RN-associated pustulosis: An evolution of acrodermatitis continua of Hallopeau to generalized pustular psoriasis.

Indian journal of dermatology, venereology and leprology
2020

Visual Dermatology: Acrodermatitis Continua of Hallopeau.

Journal of cutaneous medicine and surgery
2020

Etanercept for pediatric acrodermatitis continua of Hallopeau: A case report and literature review.

Dermatologic therapy
2020

Treatment of acrodermatitis continua of Hallopeau: A case series of 39 patients.

The Journal of dermatology
2020

Biologics in the treatment of pustular psoriasis.

Expert opinion on drug safety
2020

Clinical characteristics, genetics, comorbidities and treatment of palmoplantar pustulosis: A retrospective analysis of 66 cases in a single center in Taiwan.

The Journal of dermatology
2020

Intramatricial methotrexate for treatment of resistant acrodermatitis continua of Hallopeau: An alternative in COVID-19.

Dermatologic therapy
2020

A case of pediatric acrodermatitis continua of Hallopeau successfully treated with adalimumab biosimilar.

Dermatologic therapy
2020

Spesolimab: A Novel Treatment for Pustular Psoriasis.

Journal of cutaneous medicine and surgery
2020

Efficacy and safety of TNF blockers and of ustekinumab in palmoplantar pustulosis and in acrodermatitis continua of Hallopeau.

Journal of the European Academy of Dermatology and Venereology : JEADV
2020

Pustular Psoriasis: The Dawn of a New Era.

Acta dermato-venereologica
2020

Effectiveness of brodalumab in acrodermatitis continua of Hallopeau: A case report.

Dermatologic therapy
2020

Image Gallery: Pemphigus vulgaris mimicking acrodermatitis continua of Hallopeau.

The British journal of dermatology
2019

Treatment of Recalcitrant Acrodermatitis Continua of Hallopeau With Brodalumab.

Journal of drugs in dermatology : JDD
2019

Acrodermatitis continua of Hallopeau: clinical perspectives.

Psoriasis (Auckland, N.Z.)
2019

Case Report: Paradoxical acrodermatitis of Hallopeau-like eruption following anti-IL-17 therapy.

F1000Research
2019

Crusted scabies mimicking an acrodermatitis continua of Hallopeau.

Boletin medico del Hospital Infantil de Mexico
2019

Outcomes of various types of therapy in patients with treatment-resistant acrodermatitis continua of Hallopeau.

Biologics : targets &amp; therapy
2021

Treatment of acrodermatitis continua of hallopeau with ixekizumab.

The Journal of dermatological treatment
2019

Successful treatment of acrodermatitis continua of Hallopeau with apremilast.

The Journal of dermatology
2019

Nails as immune-privileged sites: A case of disabling Acrodermatitis continua of Hallopeau successfully treated with Apremilast.

Dermatologic therapy
2019

Biologic therapy for acrodermatitis continua of Hallopeau: Successful treatment with secukinumab and review of the literature.

Dermatologic therapy
2019

The Use of Biologic Therapy in the Treatment of Acrodermatitis Continua of Hallopeau: A Review.

Journal of cutaneous medicine and surgery
2019

Acrodermatitis Continua of Hallopeau with Psoriatic Arthritis.

The Journal of rheumatology
2019

Acrodermatitis continua of Hallopeau response to optimized biological therapy.

Dermatology online journal
2019

Acrodermatitis continua of Hallopeau successfully treated with adalimumab: A case report.

SAGE open medical case reports
2019

Apremilast as a new treatment option for Acrodermatitis continua of Hallopeau.

The Australasian journal of dermatology
2019

Acrodermatitis continua of Hallopeau with dense infiltration of IgG4-positive cells in the lesional dermis.

The British journal of dermatology
2018

Acrodermatitis Continua of Hallopeau.

JAMA dermatology
2018

Acrodermatitis continua of Hallopeau successfully treated with secukinumab.

The Journal of dermatological treatment
2018

Recalcitrant annular pustular psoriasis associated with psoriatic arthritis successfully treated with secukinumab.

JAAD case reports
2019

Clinical and genetic differences between pustular psoriasis subtypes.

The Journal of allergy and clinical immunology
2018

A case of acrodermatitis continua of Hallopeau (ACH) successfully treated with sulfasalazine.

Dermatologic therapy
2018

Acrodermatitis continua of Hallopeau and geographic tongue are variants of pustular psoriasis.

JAAD case reports
2018

Treatment of acrodermatitis continua of Hallopeau with ustekinumab as monotherapy after failure of anti-TNF agents.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2018

Generalized pustular psoriasis: a review and update on treatment.

Journal of the European Academy of Dermatology and Venereology : JEADV
2017

Acrodermatitis Continua of Hallopeau with Bone Resorption in an 8-Year-Old Patient: A Case Report.

Case reports in dermatology
2018

Pustular psoriasis and related pustular skin diseases.

The British journal of dermatology
2017

Highly Resistant Acrodermatitis Continua of Hallopeau and Pustular Psoriasis.

Skin appendage disorders
2018

Efficacy and safety of acitretin monotherapy in children with pustular psoriasis: results from 15 cases and a literature review.

The Journal of dermatological treatment
2018

The genetic basis for most patients with pustular skin disease remains elusive.

The British journal of dermatology
2017

Dermoscopy in facilitating the recognition of acrodermatitis continua of Hallopeau.

The Journal of dermatology
2017

European consensus statement on phenotypes of pustular psoriasis.

Journal of the European Academy of Dermatology and Venereology : JEADV
2017

A Painful Periungual Red Spot in a Patient with Onychodystrophy.

Skin appendage disorders
2017

Short- and Long-Term Management of an Acute Pustular Psoriasis Flare: A Case Report.

Journal of cutaneous medicine and surgery
2017

Acrodermatitis of Hallopeau and erosive oral mucositis successfully treated with secukinumab.

JAAD case reports
2018

Refractory acrodermatitis continua of Hallopeau successfully treated with oral alitretinoin.

The Journal of dermatology
2017

Ustekinumab for the treatment of acrodermatitis continua of Hallopeau refractory to anti-TNF agents.

Dermatologic therapy
2017

Novel Application of High-Dose-Rate Brachytherapy for Severe, Recalcitrant Acrodermatitis Continua of Hallopeau.

JAMA dermatology
2017

Secukinumab for Acrodermatitis Continua of Hallopeau.

JAMA dermatology
2016

Speckled variance optical coherence tomography for the assessment of nail involvement in acrodermatitis continua of Hallopeau: A case study.

The Journal of international medical research
2016

Pustular psoriasis: pathophysiology and current treatment perspectives.

Psoriasis (Auckland, N.Z.)
2017

Acrodermatitis Continua of Hallopeau with Granuloma-like Vegetation, Osteolysis and IL36RN Mutation.

Acta dermato-venereologica
2016

IL36RN Mutations Affect Protein Expression and Function: A Basis for Genotype-Phenotype Correlation in Pustular Diseases.

The Journal of investigative dermatology
2016

Pustular Psoriasis of Pregnancy with Acrodermatitis Continua of Hallopeau.

Indian journal of dermatology
2016

A Case of Acrodermatitis Continua of Hallopeau Following Chronic Pustular Cheilitis.

Dermatology and therapy
2016

[A man with a painful index finger].

Nederlands tijdschrift voor geneeskunde
2016

Correlation of IL36RN mutation with different clinical features of pustular psoriasis in Chinese patients.

Archives of dermatological research
2016

Treatment of Acrodermatitis Continua of Hallopeau With Ustekinumab as Monotherapy.

JAMA dermatology
2015

Iloprost administration in acrodermatitis of Hallopeau complicated by acquired toes syndactyly: a case report and review of the literature.

European review for medical and pharmacological sciences
2015

Successful treatment of Acrodermatitis continua of Hallopeau associated with psoriatic arthritis with adalimumab.

European journal of rheumatology
Ver todos os 176 no EuropePMC

Associações

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Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

Ainda não existe comunidade no Raras para Acrodermatite contínua de Hallopeau

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Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

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. Case Report: Periungual Xeligekimab injection for refractory Acrodermatitis continua of Hallopeau.
    Frontiers in immunology· 2026· PMID 41853264mais citado
  2. Dual IL-17A/F Blockade for Acrodermatitis Continua of Hallopeau: A Clinical Response to Bimekizumab.
    Journal of drugs in dermatology : JDD· 2026· PMID 41642132mais citado
  3. Clinical Features and Long-Term Outcomes in 109 Pediatric Generalized Pustular Psoriasis.
    Dermatology (Basel, Switzerland)· 2026· PMID 41252319mais citado
  4. Response to Han et al., "Comment on Iorizzo's acrodermatitis continua of Hallopeau-clinical review and proposed management algorithm".
    Journal of the American Academy of Dermatology· 2026· PMID 41276128mais citado
  5. Comment on Iorizzo et al, "Acrodermatitis continua of Hallopeau-clinical review and proposed management algorithm".
    Journal of the American Academy of Dermatology· 2026· PMID 41276122mais citado
  6. Clinical phenotype of 504 pustular psoriasis patients from Europe and Egypt: a multi-center observational study.
    Clin Exp Dermatol· 2025· PMID 41403177recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:163931(Orphanet)
  2. OMIM OMIM:614204(OMIM)
  3. MONDO:0013626(MONDO)
  4. GARD:17679(GARD (NIH))
  5. Variantes catalogadas(ClinVar)
  6. Busca completa no PubMed(PubMed)
  7. Q1947290(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

Acrodermatite contínua de Hallopeau
Compêndio · Raras BR

Acrodermatite contínua de Hallopeau

ORPHA:163931 · MONDO:0013626
Prevalência
1-9 / 100 000
Herança
Autosomal recessive
CID-10
L40.2 · Acrodermatite contínua
CID-11
Medicamentos
6 registrados
Início
Adult
Prevalência
6.0 (Worldwide)
MedGen
UMLS
C0392439
Repurposing
5 candidatos
adelmidrolanti-inflammatory agent
clobetasone-butyratecorticosteroid agonist
crisaborolephosphodiesterase inhibitor
+2 outros
EuropePMC
Wikidata
Papers 10a
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