Doença genética crônica rara da pele caracterizada por hiperqueratose e eritema transitório.
Introdução
O que você precisa saber de cara
Doença genética crônica rara da pele caracterizada por hiperqueratose e eritema transitório.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 19 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 67 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
8 genes identificados com associação a esta condição. Padrão de herança: Autosomal dominant, Autosomal recessive.
Structural component of the gap junction, a specialized intercellular structure consisting of a cluster of closely packed pairs of transmembrane channels, the connexons, that allow passage of small molecules and electrical signals between neighboring cells (By similarity). Forms homotypic and heterotypic channels gated by transjunctional voltage (By similarity). May play a critical role in the physiology of hearing by participating in the recycling of potassium to the cochlear endolymph (Probabl
Cell membraneCell junction, gap junctionEndoplasmic reticulumCell junction
Oculodentodigital dysplasia
A disease characterized by a typical facial appearance and variable involvement of the eyes, dentition, and fingers. Characteristic facial features include a narrow, pinched nose with hypoplastic alae nasi, prominent columella and thin anteverted nares together with a narrow nasal bridge, and prominent epicanthic folds giving the impression of hypertelorism. The teeth are usually small and carious. Typical eye findings include microphthalmia and microcornea. The characteristic digital malformation is complete syndactyly of the fourth and fifth fingers (syndactyly type III) but the third finger may be involved and associated camptodactyly is a common finding. Cardiac abnormalities are observed in rare instances.
Major keratinocyte cell envelope protein
CytoplasmNucleus, nucleoplasm
Vohwinkel syndrome with ichthyosis
A variant form of Vohwinkel syndrome without hearing loss and associated with ichthyosiform dermatosis. Clinical features include palmoplantar keratoderma, pseudoainhum and ichthyosis. Compact hyperkeratosis with round retained nuclei and hypergranulosis is observed on skin biopsies.
Erythrokeratodermia variabilis et progressiva 5
A form of erythrokeratodermia variabilis et progressiva, a genodermatosis characterized by the coexistence of two independent skin lesions: transient erythema and hyperkeratosis that is usually localized but occasionally occurs in its generalized form. Clinical presentation varies significantly within a family and from one family to another. Palmoplantar keratoderma is present in around 50% of cases. EKVP5 inheritance is autosomal recessive.
Calcium-activated selective cation channel that mediates membrane depolarization (PubMed:12015988, PubMed:12842017, PubMed:29211723, PubMed:30528822). While it is activated by increase in intracellular Ca(2+), it is impermeable to it (PubMed:12015988). Mediates transport of monovalent cations (Na(+) > K(+) > Cs(+) > Li(+)), leading to depolarize the membrane (PubMed:12015988). It thereby plays a central role in cadiomyocytes, neurons from entorhinal cortex, dorsal root and vomeronasal neurons, e
Cell membraneEndoplasmic reticulumGolgi apparatus
Progressive familial heart block 1B
A cardiac bundle branch disorder characterized by progressive alteration of cardiac conduction through the His-Purkinje system, with a pattern of a right bundle-branch block and/or left anterior hemiblock occurring individually or together. It leads to complete atrio-ventricular block causing syncope and sudden death.
Component of intercellular desmosome junctions (By similarity). Plays a role in stratified epithelial integrity and cell-cell adhesion by promoting desmosome assembly (By similarity). Thereby plays a role in barrier function of the skin against infection (By similarity). Plays a role in mammary epithelial tissue homeostasis and remodeling during and after pregnancy, potentially via its involvement in desmosome cell-cell junctions (By similarity). Required for tooth enamel development via facilit
Cell junction, desmosomeCell membraneCytoplasm
Erythrokeratodermia variabilis et progressiva 7
A form of erythrokeratodermia variabilis et progressiva, a genodermatosis characterized by the coexistence of two independent skin lesions: transient erythema and hyperkeratosis that is usually localized but occasionally occurs in its generalized form. Clinical presentation varies significantly within a family and from one family to another. Palmoplantar keratoderma is present in around 50% of cases. EKVP7 is an autosomal recessive form characterized by palmoplantar keratoderma that extends to the dorsal surface of the hands and feet, as well as erythematous annular skin lesions. Pruritus, woolly hair, and dystrophic nails may also be present.
One gap junction consists of a cluster of closely packed pairs of transmembrane channels, the connexons, through which materials of low MW diffuse from one cell to a neighboring cell
Cell membraneCell junction, gap junction
Erythrokeratodermia variabilis et progressiva 1
A form of erythrokeratodermia variabilis et progressiva, a genodermatosis characterized by the coexistence of two independent skin lesions: transient erythema and hyperkeratosis that is usually localized but occasionally occurs in its generalized form. Clinical presentation varies significantly within a family and from one family to another. Palmoplantar keratoderma is present in around 50% of cases.
Structural component of gap junctions (By similarity). Gap junctions are dodecameric channels that connect the cytoplasm of adjoining cells. They are formed by the docking of two hexameric hemichannels, one from each cell membrane (By similarity). Small molecules and ions diffuse from one cell to a neighboring cell via the central pore (By similarity)
Cell membraneCell junction, gap junction
Erythrokeratodermia variabilis et progressiva 2
A form of erythrokeratodermia variabilis et progressiva, a genodermatosis characterized by the coexistence of two independent skin lesions: transient erythema and hyperkeratosis that is usually localized but occasionally occurs in its generalized form. Clinical presentation varies significantly within a family and from one family to another. Palmoplantar keratoderma is present in around 50% of cases.
Catalyzes the reduction of 3'-oxosphinganine (3-ketodihydrosphingosine/KDS) to sphinganine (dihydrosphingosine/DHS), the second step of de novo sphingolipid biosynthesis
Endoplasmic reticulum membrane
Variantes genéticas (ClinVar)
164 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 218 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
18 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Eritroceratodermia variável tipo Mendes da Costa
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
Nenhum ensaio clínico registrado para esta condição.
Publicações mais relevantes
A novel missense mutation responsible for a patient with unique erythrokeratodermia variabilis with skin lesions in a swirling pattern.
Erythrokeratodermia variabilis et progressiva (EKVP) is a rare, inherited skin disease characterized by migratory erythematous areas and fixed hyperkeratosis plaques, which is most commonly caused by mutations in GJB3, GJB4 or GJA1. A 5-month-old male infant presented with erythematous skin lesions on the trunk and brown hyperkeratotic plaques, with a unique swirling pattern on the extremities. Whole-exome sequencing revealed a heterozygous missense mutation c.134G>C in GJB3, which has not previously been reported. PROVEAN (Protein Variation Effect Analyser) analysis revealed a score of -3.065, which was below the threshold of -2.5. In addition, the three-dimensional (3D) structure predicted that missense mutation p.Gly45Ala could compromise the 3D stability of the GJB3 protein, suggesting a deleterious effect. This novel missense mutation in GJB3 that caused EKVP with a unique swirling pattern in a Chinese family broadens the genetic and phenotypic spectrum of EKVP.
The evolving genetic landscape of ILVEN: A comprehensive review.
Advances in genomics have redefined inflammatory linear verrucous epidermal naevus (ILVEN) as a mosaic inflammatory disorder with diverse molecular drivers, enabling more precise diagnostic and therapeutic strategies. This review aimed to summarize the published data on genotype-phenotype correlations and associated targeted therapies in ILVEN and ILVEN-like disorders. A structured literature search was conducted using PubMed, Embase and Google Scholar, focusing on peer-reviewed studies describing mutations, pathomechanisms and treatment responses in ILVEN or ILVEN-mimicking dermatoses. Search terms included "ILVEN", "mosaic inflammatory dermatoses", "genetic mutations" and "targeted therapy". Multiple genes have been implicated in ILVEN. Somatic mutations in CARD14 activate NF-κB and IL-12/23/IL-17 signalling pathways and may be responsive to biologics such as secukinumab and ustekinumab. Mutations in NSDHL and PMVK, involved in cholesterol biosynthesis, are associated with ILVEN-like presentations and may respond to topical statin/cholesterol therapy. KRT10 mutations, which affect keratinocyte differentiation, show favourable responses to crisaborole and acitretin. GJA1 mutations disrupt connexin 43 function, resulting in ILVEN-Erythrokeratodermia Variabilis et Progressiva (EKVP) overlap and may respond to retinoids. HRAS mosaicism activates the RAS-MAPK pathway, supporting the theoretical use of MEK inhibitors. Recessive mosaicism in ABCA12 can produce linear, ILVEN-like lesions due to defective lipid barrier formation, with potential responsiveness to lipid-replenishing therapies and IL-17 blockade. The molecular characterization of ILVEN and related disorders supports a shift toward precision dermatology. As accessibility increases, genotype-directed therapy may replace empiric approaches with more targeted, effective and personalized management frameworks.
Erythrokeratodermia Variabilis et Progressiva 4 With KDSR Mutations: A Case Report.
A Pediatric Case of KRT2 Nonsyndromic Epidermal Differentiation Disorder With a Concurrent GJB4 Variant.
Recessive mosaicism in ABCA12 causes a unique phenotype of segmental congenital ichthyosiform erythroderma mimicking erythrokeratodermia variabilis.
We report a unique case of a 1-year-old boy presenting with nonpruritic erythematous patches and mild keratotic plaques, partially following the lines of Blaschko and mainly involving the extremities. Next-generation sequencing (NGS) revealed a heterozygous missense mutation c.4724C>T (p.Thr1575Met) and a de novo mosaic deletion mutation c.6861_6869del (p.Leu2288_Gly2290del) in the ABCA12 (NM_173076.3) gene from the DNA of the patient's blood. Even more to the point, the lesional skin showed clinical improvement after 2 weeks of moisturizing treatment. Therefore, partial encapsulation treatment, widely used to enhance the percutaneous absorption of drugs, is suitable for mosaic ichthyosis given its localized skin lesions.
Publicações recentes
Erythrokeratodermia Variabilis et Progressiva 4 With KDSR Mutations: A Case Report.
A Pediatric Case of KRT2 Nonsyndromic Epidermal Differentiation Disorder With a Concurrent GJB4 Variant.
A novel missense mutation responsible for a patient with unique erythrokeratodermia variabilis with skin lesions in a swirling pattern.
Recessive mosaicism in ABCA12 causes a unique phenotype of segmental congenital ichthyosiform erythroderma mimicking erythrokeratodermia variabilis.
The evolving genetic landscape of ILVEN: A comprehensive review.
📚 EuropePMC90 artigos no totalmostrando 71
Erythrokeratodermia Variabilis et Progressiva 4 With KDSR Mutations: A Case Report.
The Journal of dermatologyA Pediatric Case of KRT2 Nonsyndromic Epidermal Differentiation Disorder With a Concurrent GJB4 Variant.
The Journal of dermatologyA novel missense mutation responsible for a patient with unique erythrokeratodermia variabilis with skin lesions in a swirling pattern.
Clinical and experimental dermatologyRecessive mosaicism in ABCA12 causes a unique phenotype of segmental congenital ichthyosiform erythroderma mimicking erythrokeratodermia variabilis.
Dermatology reportsThe evolving genetic landscape of ILVEN: A comprehensive review.
Journal of the European Academy of Dermatology and Venereology : JEADVErythrokeratodermia Variabilis et Progressiva With Compound Heterozygous ABCA12 Variants.
The Australasian journal of dermatologyErythrokeratodermia Variabilis due to a Compound Heterozygous Variants in the NIPAL4 Gene.
Pediatric dermatologyNonsyndromic epidermal differentiation disorders: a new classification toward pathogenesis-based therapy.
The British journal of dermatologySkin disease-associated GJB4 variants differentially influence connexin stability, cell viability and channel function.
The Journal of physiologyThe genetic and molecular basis of a connexin-linked skin disease.
The Biochemical journalProtean cutaneous manifestation caused by ABCA12 variants: erythrokeratodermia variabilis-like ichthyosis and unique palmoplantar keratoderma.
Clinical and experimental dermatologyAdult-onset erythrokeratodermia variabilis (EKV) triggered by pregnancy and crash dieting: A rare case report.
JPMA. The Journal of the Pakistan Medical AssociationFour cases of Chanarin-Dorfman syndrome presenting with different types of erythrokeratoderma.
Pediatric dermatologyNovel compound heterozygous MPDU1 variants causing congenital disorders of glycosylation presenting with erythrokeratodermia variabilis.
The Journal of dermatologyPotential Clinical Benefit of Very Long Chain Fatty Acid Supplementation in Spinocerebellar Ataxia Type 34.
Cerebellum (London, England)Erythrokeratodermia Variabilis-like Phenotype in Patients Carrying ABCA12 Mutations.
GenesNovel variants in ABCA12 cause erythrokeratodermia variabilis.
The British journal of dermatologyPartial Loss of Function ABCA12 Mutations Generate Reduced Deposition of Glucosyl-Ceramide, Leading to Patchy Ichthyosis and Erythrodermia Resembling Erythrokeratodermia Variabilis et Progressiva (EKVP).
International journal of molecular sciencesA novel ELOVL4 variant, L168S, causes early childhood-onset Spinocerebellar ataxia-34 and retinal dysfunction: a case report.
Acta neuropathologica communicationsGJB4 variants linked to skin disease exhibit a trafficking deficiency en route to gap junction formation that can be restored by co-expression of select connexins.
Frontiers in cell and developmental biologyNetherton syndrome in a Bulgarian patient : Presentation of a case and an update of therapeutic options.
Wiener medizinische Wochenschrift (1946)ELOVL4 Mutations That Cause Spinocerebellar Ataxia-34 Differentially Alter Very Long Chain Fatty Acid Biosynthesis.
Journal of lipid researchIncompletely penetrant TRPM4-associated progressive symmetric erythrokeratodermia responses to methotrexate.
The Journal of dermatologyA Connexin Gene (GJB3) Mutation in a Chinese Family With Erythrokeratodermia Variabilis, Ichthyosis and Nonsyndromic Hearing Loss: Case Report and Mutations Update.
Frontiers in geneticsAnnular Epidermolytic Ichthyosis Mimicking Greither Disease: A Case Report and Literature Review.
The American journal of case reportsInterrogation of Carboxy-Terminus Localized GJA1 Variants Associated with Erythrokeratodermia Variabilis et Progressiva.
International journal of molecular sciencesErythrokeratodermia variabilis et progressiva due to a novel mutation in GJB4.
Experimental dermatologyExpanding the clinical phenotype associated with NIPAL4 mutation: Study of a Tunisian consanguineous family with erythrokeratodermia variabilis-Like Autosomal Recessive Congenital Ichthyosis.
PloS oneProgressive symmetric erythrokeratodermia with spinocerebellar ataxia due to ELOVL4 mutation in a Chinese family.
Indian journal of dermatology, venereology and leprology[Diagnosis and progress in the progressive symmetric erythrokeratodermia].
Zhonghua yi xue za zhiThe Complex and Critical Role of Glycine 12 (G12) in Beta-Connexins of Human Skin.
International journal of molecular sciencesGjb3 Gene Mutations in Non-Syndromic Hearing Loss of Bloch, Kurd, and Turkmen Ethnicities in Iran.
Iranian journal of public healthVery long chain fatty acid-containing lipids: a decade of novel insights from the study of ELOVL4.
Journal of lipid researchMEDNIK-like syndrome due to compound heterozygous mutations in AP1B1.
Journal of the European Academy of Dermatology and Venereology : JEADVAnnular epidermolytic ichthyosis: a case report and literature review.
Anais brasileiros de dermatologiaFulminant myocarditis following recurrent generalized erythrokeratoderma in a child with a heterozygous GJA1 variant.
American journal of medical genetics. Part AClinical variability of the GJB4:c.35G > A gene variant: a study of a large Brazilian erythrokeratodermia pedigree.
International journal of dermatologyCharacterization of the phenotype with cognitive impairment and protein mislocalization in SCA34.
Neurology. GeneticsErythrokeratodermia variabilis with hypertrichosis on the lesions.
Chinese medical journalErythrokeratodermia variabilis et progressiva with a rare GJB3 mutation.
The Journal of dermatologyIdentification of a CDH12 potential candidate genetic variant for an autosomal dominant form of transgrediens and progrediens palmoplantar keratoderma in a Tunisian family.
Journal of human geneticsNovel and recurrent mutations in GJB3 and GJB4 cause erythrokeratodermia variabilis et progressiva.
Indian journal of dermatology, venereology and leprologyCase of erythrokeratodermia variabilis successfully treated with narrowband ultraviolet B.
The Journal of dermatologyThe Role of Desmoglein 1 in Gap Junction Turnover Revealed through the Study of SAM Syndrome.
The Journal of investigative dermatologyA Case of Erythrokeratodermia Variabilis in Korean.
Annals of dermatology[Progressive symmetric erythrokeratodermia: Activating mutations of TRPM4].
Annales de dermatologie et de venereologieRecessive mosaicism in ABCA12 causes blaschkoid congenital ichthyosiform erythroderma.
The British journal of dermatologyPluripotent stem cell-derived bile canaliculi-forming hepatocytes to study genetic liver diseases involving hepatocyte polarity.
Journal of hepatologyTwo de novo GJA1 mutation in two sporadic patients with erythrokeratodermia variabilis et progressiva.
Molecular genetics & genomic medicineChronic symmetrically distributed hyperpigmented plaques in a middle-age woman.
JAAD case reportsConnexin43 mutations linked to skin disease have augmented hemichannel activity.
Scientific reportsA heterozygous mutation in GJA1 gene in Chinese family with serious erythrokeratodermia variabilis et progressive.
Chinese medical journalA20 and ABIN1 Suppression of a Keratinocyte Inflammatory Program with a Shared Single-Cell Expression Signature in Diverse Human Rashes.
The Journal of investigative dermatologyGain-of-Function Mutations in TRPM4 Activation Gate Cause Progressive Symmetric Erythrokeratodermia.
The Journal of investigative dermatologySphingolipid dysregulation due to lack of functional KDSR impairs proplatelet formation causing thrombocytopenia.
HaematologicaA p.478I>T KRT1 mutation in a case of annular epidermolytic ichthyosis.
Pediatric dermatologyExome sequencing identifies novel compound heterozygous mutations in GJB3 gene that cause erythrokeratodermia variabilis et progressiva.
The Australasian journal of dermatologyA rare missense mutation in GJB3 (Cx31G45E) is associated with a unique cellular phenotype resulting in necrotic cell death.
Experimental dermatologyExome Sequencing Identifies a Novel Nonsense Mutation of MYO6 as the Cause of Deafness in a Brazilian Family.
Annals of human geneticsRecessive progressive symmetric erythrokeratoderma results from a homozygous loss-of-function mutation of KRT83 and is allelic with dominant monilethrix.
Journal of medical geneticsInflammatory Linear Verrucous Epidermal Nevus with a Postzygotic GJA1 Mutation Is a Mosaic Erythrokeratodermia Variabilis et Progressiva.
The Journal of investigative dermatologyStriate Palmoplantar Keratoderma Showing Transgrediens in a Patient Harbouring Heterozygous Nonsense Mutations in Both DSG1 and SERPINB7.
Acta dermato-venereologicaErythrokeratodermia variabilis et progressiva.
The Journal of dermatologyJapanese sporadic case of erythrokeratodermia variabilis caused by the connexin-30.3 (GJB4) mutation: Is Glycine 12 a mutational hotspot in the connexin family?
The Journal of dermatologyHerpes simplex virus in erythrokeratoderma variabilis.
Dermatology online journalErythrokeratoderma Variabilis Caused by p.Gly45Glu in Connexin 31: Importance of the First Extracellular Loop Glycine Residue for Gap Junction Function.
Acta dermato-venereologicaCase of erythrokeratodermia variabilis successfully treated with oral vitamin A.
The Journal of dermatologyPathogenic Cx31 is un/misfolded to cause skin abnormality via a Fos/JunB-mediated mechanism.
Human molecular geneticsA Novel Mutation in ELOVL4 Leading to Spinocerebellar Ataxia (SCA) With the Hot Cross Bun Sign but Lacking Erythrokeratodermia: A Broadened Spectrum of SCA34.
JAMA neurologyErythrokeratodermia variabilis et progressiva allelic to oculo-dento-digital dysplasia.
The Journal of investigative dermatologyThe novel GJB3 mutation p.Thr202Asn in the M4 transmembrane domain underlies erythrokeratodermia variabilis.
The British journal of dermatologyAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Eritroceratodermia variável tipo Mendes da Costa.
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Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
Ainda não existe comunidade no Raras para Eritroceratodermia variável tipo Mendes da Costa
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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.
- A novel missense mutation responsible for a patient with unique erythrokeratodermia variabilis with skin lesions in a swirling pattern.
- The evolving genetic landscape of ILVEN: A comprehensive review.Journal of the European Academy of Dermatology and Venereology : JEADV· 2026· PMID 41065394mais citado
- Erythrokeratodermia Variabilis et Progressiva 4 With KDSR Mutations: A Case Report.
- A Pediatric Case of KRT2 Nonsyndromic Epidermal Differentiation Disorder With a Concurrent GJB4 Variant.
- Recessive mosaicism in ABCA12 causes a unique phenotype of segmental congenital ichthyosiform erythroderma mimicking erythrokeratodermia variabilis.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:317(Orphanet)
- MONDO:0017851(MONDO)
- GARD:16528(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q3591493(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