Raras
Buscar doenças, sintomas, genes...
Eritroceratodermia variável tipo Mendes da Costa
ORPHA:317CID-10 · Q82.8CID-11 · EC20.0YDOENÇA RARA

Doença genética crônica rara da pele caracterizada por hiperqueratose e eritema transitório.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

Doença genética crônica rara da pele caracterizada por hiperqueratose e eritema transitório.

Publicações científicas
138 artigos
Último publicado: 2026 Apr

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
<1 / 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
200
pacientes catalogados
Início
Infancy
+ neonatal
🏥
SUS: Cobertura mínimaScore: 15%
CID-10: Q82.8
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (5)
0202010503
Cariótipo — bandas G, Q ou Rgenetic_test
0202010600
Pesquisa de microdeleções/microduplicações por FISHlab_test
0202010694
Sequenciamento completo do exoma (WES)rehabilitation
0202010260
Dosagem de alfa-fetoproteína
0301070040
Atendimento em reabilitação — doenças raras
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

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Sinais e sintomas

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

Partes do corpo afetadas

🧬
Pele e cabelo
26 sintomas
🦴
Ossos e articulações
4 sintomas
❤️
Coração
3 sintomas
👁️
Olhos
3 sintomas
💪
Músculos
3 sintomas
👂
Ouvidos
2 sintomas

+ 19 sintomas em outras categorias

Características mais comuns

90%prev.
Hiperceratose
Muito frequente (99-80%)
90%prev.
Erupção cutânea
Muito frequente (99-80%)
90%prev.
Mácula hiperpigmentada
Muito frequente (99-80%)
90%prev.
Fotossensibilidade cutânea
Muito frequente (99-80%)
90%prev.
Microcefalia
Muito frequente (99-80%)
90%prev.
Mácula
Muito frequente (99-80%)
67sintomas
Muito frequente (10)
Frequente (8)
Ocasional (11)
Sem dados (38)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 67 características clínicas mais associadas, ordenadas por frequência.

HiperceratoseHyperkeratosis
Muito frequente (99-80%)90%
Erupção cutâneaSkin rash
Muito frequente (99-80%)90%
Mácula hiperpigmentadaHypermelanotic macule
Muito frequente (99-80%)90%
Fotossensibilidade cutâneaCutaneous photosensitivity
Muito frequente (99-80%)90%
MicrocefaliaMicrocephaly
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico138PubMed
Últimos 10 anos71publicações
Pico201912 papers
Linha do tempo
2026Hoje · 2026📈 2019Ano 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

8 genes identificados com associação a esta condição. Padrão de herança: Autosomal dominant, Autosomal recessive.

GJA1Gap junction alpha-1 proteinDisease-causing germline mutation(s) inAltamente restrito
FUNÇÃO

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

LOCALIZAÇÃO

Cell membraneCell junction, gap junctionEndoplasmic reticulumCell junction

VIAS BIOLÓGICAS (4)
Regulation of gap junction activitySARS-CoV-2 targets PDZ proteins in cell-cell junctionGap junction assemblyMicrotubule-dependent trafficking of connexons from Golgi to the plasma membrane
MECANISMO DE DOENÇA

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.

EXPRESSÃO TECIDUAL(Ubíquo)
Skin Not Sun Exposed Suprapubic
485.1 TPM
Glândula adrenal
439.8 TPM
Skin Sun Exposed Lower leg
408.1 TPM
Aorta
387.9 TPM
Cervix Endocervix
368.9 TPM
OUTRAS DOENÇAS (10)
oculodentodigital dysplasiaoculodentodigital dysplasia, autosomal recessiveautosomal dominant palmoplantar keratoderma and congenital alopeciacraniometaphyseal dysplasia, autosomal recessive
HGNC:4274UniProt:P17302
LORICRINLoricrinCandidate gene tested inTolerante
FUNÇÃO

Major keratinocyte cell envelope protein

LOCALIZAÇÃO

CytoplasmNucleus, nucleoplasm

VIAS BIOLÓGICAS (2)
Formation of the cornified envelopeDifferentiation of Keratinocytes in Interfollicular Epidermis in Mammalian Skin
MECANISMO DE DOENÇA

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.

OUTRAS DOENÇAS (2)
loricrin keratodermaerythrokeratodermia variabilis
HGNC:6663UniProt:P23490
KRT83Keratin, type II cuticular Hb3Disease-causing germline mutation(s) inTolerante
LOCALIZAÇÃO

VIAS BIOLÓGICAS (2)
KeratinizationFormation of the cornified envelope
MECANISMO DE DOENÇA

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.

EXPRESSÃO TECIDUAL(Baixa expressão)
Brain Spinal cord cervical c-1
1.8 TPM
Tireoide
1.5 TPM
Córtex cerebral
0.7 TPM
Brain Frontal Cortex BA9
0.6 TPM
Substância negra
0.6 TPM
INTERAÇÕES PROTEICAS (2)
OUTRAS DOENÇAS (4)
monilethrix-3erythrokeratodermia variabilis et progressiva 5monilethrixerythrokeratodermia variabilis
HGNC:6460UniProt:P78385
TRPM4Transient receptor potential cation channel subfamily M member 4Disease-causing germline mutation(s) inTolerante
FUNÇÃO

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

LOCALIZAÇÃO

Cell membraneEndoplasmic reticulumGolgi apparatus

VIAS BIOLÓGICAS (2)
TRP channelsSensory perception of sweet, bitter, and umami (glutamate) taste
MECANISMO DE DOENÇA

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.

EXPRESSÃO TECIDUAL(Ubíquo)
Cólon transverso
69.4 TPM
Próstata
61.3 TPM
Skin Sun Exposed Lower leg
47.5 TPM
Glândula salivar
45.2 TPM
Skin Not Sun Exposed Suprapubic
41.8 TPM
OUTRAS DOENÇAS (5)
progressive familial heart block type IBerythrokeratodermia variabilis et progressiva 6Brugada syndromeprogressive familial heart block
HGNC:17993UniProt:Q8TD43
PERPp53 apoptosis effector related to PMP-22Disease-causing germline mutation(s) inTolerante
FUNÇÃO

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

LOCALIZAÇÃO

Cell junction, desmosomeCell membraneCytoplasm

VIAS BIOLÓGICAS (2)
TP53 regulates transcription of several additional cell death genes whose specific roles in p53-dependent apoptosis remain uncertainFormation of the cornified envelope
MECANISMO DE DOENÇA

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.

EXPRESSÃO TECIDUAL(Ubíquo)
Skin Sun Exposed Lower leg
2080.4 TPM
Skin Not Sun Exposed Suprapubic
1935.4 TPM
Esôfago - Mucosa
1234.2 TPM
Vagina
815.2 TPM
Glândula salivar
336.6 TPM
OUTRAS DOENÇAS (3)
Olmsted syndrome 2erythrokeratodermia variabilis et progressiva 7Olmsted syndrome
HGNC:17637UniProt:Q96FX8
GJB3Gap junction beta-3 proteinDisease-causing germline mutation(s) inTolerante
FUNÇÃO

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

LOCALIZAÇÃO

Cell membraneCell junction, gap junction

VIAS BIOLÓGICAS (1)
Gap junction assembly
MECANISMO DE DOENÇA

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.

EXPRESSÃO TECIDUAL(Tecido-específico)
Skin Sun Exposed Lower leg
184.0 TPM
Skin Not Sun Exposed Suprapubic
182.9 TPM
Esôfago - Mucosa
87.3 TPM
Vagina
41.6 TPM
Próstata
4.5 TPM
INTERAÇÕES PROTEICAS (5)
OUTRAS DOENÇAS (7)
autosomal dominant nonsyndromic hearing loss 2Bautosomal recessive nonsyndromic hearing loss 1Aerythrokeratodermia variabilis et progressiva 1hearing loss, autosomal recessive
HGNC:4285UniProt:O75712
GJB4Gap junction beta-4 proteinDisease-causing germline mutation(s) inDesconhecido
FUNÇÃO

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)

LOCALIZAÇÃO

Cell membraneCell junction, gap junction

VIAS BIOLÓGICAS (1)
Gap junction assembly
MECANISMO DE DOENÇA

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.

VIAS REACTOME (1)
EXPRESSÃO TECIDUAL(Tecido-específico)
Skin Not Sun Exposed Suprapubic
44.3 TPM
Skin Sun Exposed Lower leg
43.6 TPM
Esôfago - Mucosa
2.4 TPM
Vagina
2.1 TPM
Próstata
0.9 TPM
OUTRAS DOENÇAS (2)
erythrokeratodermia variabilis et progressiva 2erythrokeratodermia variabilis
HGNC:4286UniProt:Q9NTQ9
KDSR3-ketodihydrosphingosine reductaseDisease-causing germline mutation(s) (loss of function) inTolerante
FUNÇÃO

Catalyzes the reduction of 3'-oxosphinganine (3-ketodihydrosphingosine/KDS) to sphinganine (dihydrosphingosine/DHS), the second step of de novo sphingolipid biosynthesis

LOCALIZAÇÃO

Endoplasmic reticulum membrane

VIAS BIOLÓGICAS (1)
Sphingolipid de novo biosynthesis
EXPRESSÃO TECIDUAL(Ubíquo)
Nervo tibial
48.9 TPM
Aorta
44.3 TPM
Skin Sun Exposed Lower leg
42.7 TPM
Brain Spinal cord cervical c-1
40.2 TPM
Artéria tibial
40.0 TPM
OUTRAS DOENÇAS (2)
erythrokeratodermia variabilis et progressiva 4erythrokeratodermia variabilis
HGNC:4021UniProt:Q06136

Variantes genéticas (ClinVar)

164 variantes patogênicas registradas no ClinVar.

🧬 GJA1: NM_000165.5(GJA1):c.460A>C (p.Thr154Pro) ()
🧬 GJA1: NM_000165.5(GJA1):c.442C>G (p.Arg148Gly) ()
🧬 GJA1: NM_000165.5(GJA1):c.402G>T (p.Lys134Asn) ()
🧬 GJA1: GRCh37/hg19 6q22.1-22.33(chr6:114742335-127346798)x1 ()
🧬 GJA1: NM_000165.5(GJA1):c.1131TGA[3] (p.Asp379_Leu380insAsp) ()
Ver todas no ClinVar

Classificação de variantes (ClinVar)

Distribuição de 218 variantes classificadas pelo ClinVar.

11
207
Patogênica (5.0%)
VUS (95.0%)
VARIANTES MAIS SIGNIFICATIVAS
KDSR: NM_002035.4(KDSR):c.190C>T (p.Arg64Ter) [Likely pathogenic]
GJA1: NM_000165.5(GJA1):c.488C>G (p.Ser163Cys) [Uncertain significance]
GJB4: NM_153212.3(GJB4):c.199G>A (p.Glu67Lys) [Uncertain significance]
GJB3: NM_024009.3(GJB3):c.50C>A (p.Ser17Tyr) [Uncertain significance]
KRT83: NM_002282.3(KRT83):c.72_78del (p.Pro25fs) [Uncertain significance]

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Eritroceratodermia variável tipo Mendes da Costa

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Selecione um estado ou use sua localização para ver resultados.

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Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

Pesquisa e ensaios clínicos

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Publicações mais relevantes

Timeline de publicações
57 papers (10 anos)
#1

A novel missense mutation responsible for a patient with unique erythrokeratodermia variabilis with skin lesions in a swirling pattern.

Clinical and experimental dermatology2026 Feb 24

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.

#2

The evolving genetic landscape of ILVEN: A comprehensive review.

Journal of the European Academy of Dermatology and Venereology : JEADV2026 Mar

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.

#3

Erythrokeratodermia Variabilis et Progressiva 4 With KDSR Mutations: A Case Report.

The Journal of dermatology2026 Mar 11
#4

A Pediatric Case of KRT2 Nonsyndromic Epidermal Differentiation Disorder With a Concurrent GJB4 Variant.

The Journal of dermatology2026 Jan 27
#5

Recessive mosaicism in ABCA12 causes a unique phenotype of segmental congenital ichthyosiform erythroderma mimicking erythrokeratodermia variabilis.

Dermatology reports2025 Nov 03

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

Ver todas no PubMed

📚 EuropePMC90 artigos no totalmostrando 71

2026

Erythrokeratodermia Variabilis et Progressiva 4 With KDSR Mutations: A Case Report.

The Journal of dermatology
2026

A Pediatric Case of KRT2 Nonsyndromic Epidermal Differentiation Disorder With a Concurrent GJB4 Variant.

The Journal of dermatology
2026

A novel missense mutation responsible for a patient with unique erythrokeratodermia variabilis with skin lesions in a swirling pattern.

Clinical and experimental dermatology
2025

Recessive mosaicism in ABCA12 causes a unique phenotype of segmental congenital ichthyosiform erythroderma mimicking erythrokeratodermia variabilis.

Dermatology reports
2026

The evolving genetic landscape of ILVEN: A comprehensive review.

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

Erythrokeratodermia Variabilis et Progressiva With Compound Heterozygous ABCA12 Variants.

The Australasian journal of dermatology
2025

Erythrokeratodermia Variabilis due to a Compound Heterozygous Variants in the NIPAL4 Gene.

Pediatric dermatology
2025

Nonsyndromic epidermal differentiation disorders: a new classification toward pathogenesis-based therapy.

The British journal of dermatology
2025

Skin disease-associated GJB4 variants differentially influence connexin stability, cell viability and channel function.

The Journal of physiology
2024

The genetic and molecular basis of a connexin-linked skin disease.

The Biochemical journal
2025

Protean cutaneous manifestation caused by ABCA12 variants: erythrokeratodermia variabilis-like ichthyosis and unique palmoplantar keratoderma.

Clinical and experimental dermatology
2024

Adult-onset erythrokeratodermia variabilis (EKV) triggered by pregnancy and crash dieting: A rare case report.

JPMA. The Journal of the Pakistan Medical Association
2024

Four cases of Chanarin-Dorfman syndrome presenting with different types of erythrokeratoderma.

Pediatric dermatology
2025

Novel compound heterozygous MPDU1 variants causing congenital disorders of glycosylation presenting with erythrokeratodermia variabilis.

The Journal of dermatology
2024

Potential Clinical Benefit of Very Long Chain Fatty Acid Supplementation in Spinocerebellar Ataxia Type 34.

Cerebellum (London, England)
2024

Erythrokeratodermia Variabilis-like Phenotype in Patients Carrying ABCA12 Mutations.

Genes
2024

Novel variants in ABCA12 cause erythrokeratodermia variabilis.

The British journal of dermatology
2023

Partial 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 sciences
2023

A novel ELOVL4 variant, L168S, causes early childhood-onset Spinocerebellar ataxia-34 and retinal dysfunction: a case report.

Acta neuropathologica communications
2023

GJB4 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 biology
2023

Netherton syndrome in a Bulgarian patient : Presentation of a case and an update of therapeutic options.

Wiener medizinische Wochenschrift (1946)
2023

ELOVL4 Mutations That Cause Spinocerebellar Ataxia-34 Differentially Alter Very Long Chain Fatty Acid Biosynthesis.

Journal of lipid research
2022

Incompletely penetrant TRPM4-associated progressive symmetric erythrokeratodermia responses to methotrexate.

The Journal of dermatology
2022

A Connexin Gene (GJB3) Mutation in a Chinese Family With Erythrokeratodermia Variabilis, Ichthyosis and Nonsyndromic Hearing Loss: Case Report and Mutations Update.

Frontiers in genetics
2022

Annular Epidermolytic Ichthyosis Mimicking Greither Disease: A Case Report and Literature Review.

The American journal of case reports
2022

Interrogation of Carboxy-Terminus Localized GJA1 Variants Associated with Erythrokeratodermia Variabilis et Progressiva.

International journal of molecular sciences
2022

Erythrokeratodermia variabilis et progressiva due to a novel mutation in GJB4.

Experimental dermatology
2021

Expanding the clinical phenotype associated with NIPAL4 mutation: Study of a Tunisian consanguineous family with erythrokeratodermia variabilis-Like Autosomal Recessive Congenital Ichthyosis.

PloS one
2021

Progressive symmetric erythrokeratodermia with spinocerebellar ataxia due to ELOVL4 mutation in a Chinese family.

Indian journal of dermatology, venereology and leprology
2021

[Diagnosis and progress in the progressive symmetric erythrokeratodermia].

Zhonghua yi xue za zhi
2021

The Complex and Critical Role of Glycine 12 (G12) in Beta-Connexins of Human Skin.

International journal of molecular sciences
2020

Gjb3 Gene Mutations in Non-Syndromic Hearing Loss of Bloch, Kurd, and Turkmen Ethnicities in Iran.

Iranian journal of public health
2021

Very long chain fatty acid-containing lipids: a decade of novel insights from the study of ELOVL4.

Journal of lipid research
2021

MEDNIK-like syndrome due to compound heterozygous mutations in AP1B1.

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

Annular epidermolytic ichthyosis: a case report and literature review.

Anais brasileiros de dermatologia
2020

Fulminant myocarditis following recurrent generalized erythrokeratoderma in a child with a heterozygous GJA1 variant.

American journal of medical genetics. Part A
2020

Clinical variability of the GJB4:c.35G > A gene variant: a study of a large Brazilian erythrokeratodermia pedigree.

International journal of dermatology
2020

Characterization of the phenotype with cognitive impairment and protein mislocalization in SCA34.

Neurology. Genetics
2020

Erythrokeratodermia variabilis with hypertrichosis on the lesions.

Chinese medical journal
2020

Erythrokeratodermia variabilis et progressiva with a rare GJB3 mutation.

The Journal of dermatology
2020

Identification 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 genetics
2020

Novel and recurrent mutations in GJB3 and GJB4 cause erythrokeratodermia variabilis et progressiva.

Indian journal of dermatology, venereology and leprology
2020

Case of erythrokeratodermia variabilis successfully treated with narrowband ultraviolet B.

The Journal of dermatology
2020

The Role of Desmoglein 1 in Gap Junction Turnover Revealed through the Study of SAM Syndrome.

The Journal of investigative dermatology
2019

A Case of Erythrokeratodermia Variabilis in Korean.

Annals of dermatology
2019

[Progressive symmetric erythrokeratodermia: Activating mutations of TRPM4].

Annales de dermatologie et de venereologie
2020

Recessive mosaicism in ABCA12 causes blaschkoid congenital ichthyosiform erythroderma.

The British journal of dermatology
2019

Pluripotent stem cell-derived bile canaliculi-forming hepatocytes to study genetic liver diseases involving hepatocyte polarity.

Journal of hepatology
2019

Two de novo GJA1 mutation in two sporadic patients with erythrokeratodermia variabilis et progressiva.

Molecular genetics &amp; genomic medicine
2019

Chronic symmetrically distributed hyperpigmented plaques in a middle-age woman.

JAAD case reports
2019

Connexin43 mutations linked to skin disease have augmented hemichannel activity.

Scientific reports
2019

A heterozygous mutation in GJA1 gene in Chinese family with serious erythrokeratodermia variabilis et progressive.

Chinese medical journal
2019

A20 and ABIN1 Suppression of a Keratinocyte Inflammatory Program with a Shared Single-Cell Expression Signature in Diverse Human Rashes.

The Journal of investigative dermatology
2019

Gain-of-Function Mutations in TRPM4 Activation Gate Cause Progressive Symmetric Erythrokeratodermia.

The Journal of investigative dermatology
2019

Sphingolipid dysregulation due to lack of functional KDSR impairs proplatelet formation causing thrombocytopenia.

Haematologica
2018

A p.478I>T KRT1 mutation in a case of annular epidermolytic ichthyosis.

Pediatric dermatology
2019

Exome sequencing identifies novel compound heterozygous mutations in GJB3 gene that cause erythrokeratodermia variabilis et progressiva.

The Australasian journal of dermatology
2019

A rare missense mutation in GJB3 (Cx31G45E) is associated with a unique cellular phenotype resulting in necrotic cell death.

Experimental dermatology
2018

Exome Sequencing Identifies a Novel Nonsense Mutation of MYO6 as the Cause of Deafness in a Brazilian Family.

Annals of human genetics
2017

Recessive progressive symmetric erythrokeratoderma results from a homozygous loss-of-function mutation of KRT83 and is allelic with dominant monilethrix.

Journal of medical genetics
2017

Inflammatory Linear Verrucous Epidermal Nevus with a Postzygotic GJA1 Mutation Is a Mosaic Erythrokeratodermia Variabilis et Progressiva.

The Journal of investigative dermatology
2017

Striate Palmoplantar Keratoderma Showing Transgrediens in a Patient Harbouring Heterozygous Nonsense Mutations in Both DSG1 and SERPINB7.

Acta dermato-venereologica
2016

Erythrokeratodermia variabilis et progressiva.

The Journal of dermatology
2016

Japanese 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 dermatology
2016

Herpes simplex virus in erythrokeratoderma variabilis.

Dermatology online journal
2016

Erythrokeratoderma Variabilis Caused by p.Gly45Glu in Connexin 31: Importance of the First Extracellular Loop Glycine Residue for Gap Junction Function.

Acta dermato-venereologica
2015

Case of erythrokeratodermia variabilis successfully treated with oral vitamin A.

The Journal of dermatology
2015

Pathogenic Cx31 is un/misfolded to cause skin abnormality via a Fos/JunB-mediated mechanism.

Human molecular genetics
2015

A Novel Mutation in ELOVL4 Leading to Spinocerebellar Ataxia (SCA) With the Hot Cross Bun Sign but Lacking Erythrokeratodermia: A Broadened Spectrum of SCA34.

JAMA neurology
2015

Erythrokeratodermia variabilis et progressiva allelic to oculo-dento-digital dysplasia.

The Journal of investigative dermatology
2015

The novel GJB3 mutation p.Thr202Asn in the M4 transmembrane domain underlies erythrokeratodermia variabilis.

The British journal of dermatology
Ver todos os 90 no EuropePMC

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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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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. A novel missense mutation responsible for a patient with unique erythrokeratodermia variabilis with skin lesions in a swirling pattern.
    Clinical and experimental dermatology· 2026· PMID 41296677mais citado
  2. The evolving genetic landscape of ILVEN: A comprehensive review.
    Journal of the European Academy of Dermatology and Venereology : JEADV· 2026· PMID 41065394mais citado
  3. Erythrokeratodermia Variabilis et Progressiva 4 With KDSR Mutations: A Case Report.
    The Journal of dermatology· 2026· PMID 41814845mais citado
  4. A Pediatric Case of KRT2 Nonsyndromic Epidermal Differentiation Disorder With a Concurrent GJB4 Variant.
    The Journal of dermatology· 2026· PMID 41589029mais citado
  5. Recessive mosaicism in ABCA12 causes a unique phenotype of segmental congenital ichthyosiform erythroderma mimicking erythrokeratodermia variabilis.
    Dermatology reports· 2025· PMID 41186431mais citado

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:317(Orphanet)
  2. MONDO:0017851(MONDO)
  3. GARD:16528(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. 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

Compêndio · Raras BR

Eritroceratodermia variável tipo Mendes da Costa

ORPHA:317 · MONDO:0017851
Prevalência
<1 / 1 000 000
Casos
200 casos conhecidos
Herança
Autosomal dominant, Autosomal recessive
CID-10
Q82.8 · Outras malformações congênitas especificadas da pele
CID-11
Início
Infancy, Neonatal
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0265961
EuropePMC
Wikidata
Papers 10a
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