Raras
Buscar doenças, sintomas, genes...
Síndrome KID
ORPHA:477CID-10 · Q80.8CID-11 · LD27.2DOENÇA RARA

A síndrome KID/HID (Ceratite, Ictiose e Surdez, com aspecto de pele que lembra espinhos ou ouriços) é uma doença congênita rara (presente desde o nascimento) que afeta o ectoderma, um tipo de tecido que forma, entre outras coisas, a pele e os ouvidos. Ela se manifesta por: ceratite vascularizante (inflamação da córnea, a parte transparente do olho, com crescimento de vasos sanguíneos no local), lesões de pele hiperceratóticas (áreas ásperas e grossas na pele) e perda de audição.

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Introdução

O que você precisa saber de cara

📋

A síndrome KID/HID (Ceratite, Ictiose e Surdez, com aspecto de pele que lembra espinhos ou ouriços) é uma doença congênita rara (presente desde o nascimento) que afeta o ectoderma, um tipo de tecido que forma, entre outras coisas, a pele e os ouvidos. Ela se manifesta por: ceratite vascularizante (inflamação da córnea, a parte transparente do olho, com crescimento de vasos sanguíneos no local), lesões de pele hiperceratóticas (áreas ásperas e grossas na pele) e perda de audição.

Publicações científicas
236 artigos
Último publicado: 2026 Feb 26

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
100
pacientes catalogados
Início
Neonatal
🏥
SUS: Cobertura mínimaScore: 15%
CID-10: Q80.8
🇧🇷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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Sinais e sintomas

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

Partes do corpo afetadas

🧬
Pele e cabelo
22 sintomas
👁️
Olhos
13 sintomas
🧠
Neurológico
6 sintomas
🦴
Ossos e articulações
5 sintomas
👂
Ouvidos
4 sintomas
💪
Músculos
3 sintomas

+ 33 sintomas em outras categorias

Características mais comuns

90%prev.
Neovascularização corneana
Muito frequente (99-80%)
55%prev.
Hiperceratose folicular
Frequente (79-30%)
55%prev.
Infecções cutâneas recorrentes
Frequente (79-30%)
55%prev.
Deficiência auditiva neurossensorial grave
Frequente (79-30%)
55%prev.
Deficiência auditiva neurossensorial pré-lingual
Frequente (79-30%)
55%prev.
Perda visual
Frequente (79-30%)
97sintomas
Muito frequente (1)
Frequente (24)
Ocasional (14)
Muito raro (21)
Sem dados (37)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 97 características clínicas mais associadas, ordenadas por frequência.

Neovascularização corneanaCorneal neovascularization
Muito frequente (99-80%)90%
Hiperceratose folicularFollicular hyperkeratosis
Frequente (79-30%)55%
Infecções cutâneas recorrentesRecurrent skin infections
Frequente (79-30%)55%
Deficiência auditiva neurossensorial graveSevere sensorineural hearing impairment
Frequente (79-30%)55%
Deficiência auditiva neurossensorial pré-lingualPrelingual sensorineural hearing impairment
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico236PubMed
Últimos 10 anos94publicações
Pico202518 papers
Linha do tempo
2026Hoje · 2026🧪 1967Primeiro 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

3 genes identificados com associação a esta condição. Padrão de herança: Autosomal dominant, Autosomal recessive, Not applicable.

GJB2Gap junction beta-2 proteinDisease-causing germline mutation(s) inTolerante
FUNÇÃO

Structural component of gap junctions (PubMed:16849369, PubMed:17551008, PubMed:19340074, PubMed:19384972, PubMed:21094651, PubMed:26753910). 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 (PubMed:17551008, PubMed:19340074, PubMed:21094651, PubMed:26753910). Small molecules and ions diffuse from one cell to a neighboring cell via the central pore (PubMed:16849369, PubM

LOCALIZAÇÃO

Cell membraneCell junction, gap junction

VIAS BIOLÓGICAS (3)
Oligomerization of connexins into connexonsTransport of connexins along the secretory pathwayTransport of connexons to the plasma membrane
MECANISMO DE DOENÇA

Deafness, autosomal recessive, 1A

A form of non-syndromic sensorineural hearing loss. Sensorineural deafness results from damage to the neural receptors of the inner ear, the nerve pathways to the brain, or the area of the brain that receives sound information.

EXPRESSÃO TECIDUAL(Ubíquo)
Esôfago - Mucosa
1032.4 TPM
Vagina
934.9 TPM
Skin Not Sun Exposed Suprapubic
76.3 TPM
Skin Sun Exposed Lower leg
75.7 TPM
Glândula salivar
21.6 TPM
OUTRAS DOENÇAS (12)
palmoplantar keratoderma-deafness syndromeichthyosis, hystrix-like, with hearing losskeratoderma hereditarium mutilansautosomal dominant keratitis-ichthyosis-hearing loss syndrome
HGNC:4284UniProt:P29033
AP1B1AP-1 complex subunit beta-1Disease-causing germline mutation(s) inAltamente restrito
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 (PubMed:31630791). 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

LOCALIZAÇÃO

Golgi apparatusCytoplasmic vesicle, clathrin-coated vesicle membrane

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

Keratitis-ichthyosis-deafness syndrome, autosomal recessive

An autosomal recessive form of keratitis-ichthyosis-deafness syndrome, a disease characterized by the association of hyperkeratotic skin lesions with vascularizing keratitis and profound sensorineural hearing loss. KIDAR patients manifest ichthyosis, failure to thrive and developmental delay in childhood, thrombocytopenia, photophobia, and progressive hearing loss. Low plasma copper and ceruloplasmin levels have been reported in some patients.

OUTRAS DOENÇAS (2)
ichthyosiform erythroderma, corneal involvement, and hearing lossMEDNIK syndrome
HGNC:554UniProt:Q10567
GJB6Gap junction beta-6 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

Ectodermal dysplasia 2, Clouston type

A form of ectodermal dysplasia, a heterogeneous group of disorders due to abnormal development of two or more ectodermal structures such as hair, teeth, nails and sweat glands, with or without any additional clinical sign. Each combination of clinical features represents a different type of ectodermal dysplasia. ECTD2 is an autosomal dominant condition characterized by atrichosis, nail hypoplasia and deformities, hyperpigmentation of the skin, normal teeth, normal sweat and sebaceous gland function. Palmoplantar hyperkeratosis is a frequent feature. Hearing impairment has been detected in few cases.

VIAS REACTOME (1)
EXPRESSÃO TECIDUAL(Tecido-específico)
Esôfago - Mucosa
199.5 TPM
Vagina
198.2 TPM
Skin Sun Exposed Lower leg
51.2 TPM
Córtex cerebral
51.0 TPM
Skin Not Sun Exposed Suprapubic
48.1 TPM
OUTRAS DOENÇAS (7)
autosomal recessive nonsyndromic hearing loss 1Bautosomal recessive nonsyndromic hearing loss 1AClouston syndromeautosomal dominant nonsyndromic hearing loss 3B
HGNC:4288UniProt:O95452

Variantes genéticas (ClinVar)

573 variantes patogênicas registradas no ClinVar.

🧬 GJB2: NM_004004.6(GJB2):c.41A>T (p.Asn14Ile) ()
🧬 GJB2: NM_004004.6(GJB2):c.160A>G (p.Asn54Asp) ()
🧬 GJB2: NM_004004.6(GJB2):c.580T>C (p.Phe194Leu) ()
🧬 GJB2: NM_004004.6(GJB2):c.300T>G (p.His100Gln) ()
🧬 GJB2: NM_004004.6(GJB2):c.217C>T (p.His73Tyr) ()
Ver todas no ClinVar

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

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
3Fase 31
2Fase 22
1Fase 11
·Pré-clínico1
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 5 ensaios
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Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Síndrome KID

🗺️

Selecione um estado ou use sua localização para ver resultados.

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

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

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Outros ensaios clínicos

0 ensaios clínicos encontrados.

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

📖Melhor nível de evidência: Revisão
Timeline de publicações
92 papers (10 anos)
#1

Successful Use of Adalimumab for Dissecting Cellulitis in a Pediatric Patient With Keratitis-Ichthyosis-Deafness (KID) Syndrome.

Pediatric dermatology2026 Feb 09

Dissecting cellulitis of the scalp (DCS) is a chronic inflammatory condition characterized by painful, draining nodules and progressive, scarring alopecia. It is often refractory to treatment, and there are currently no FDA-approved therapies. We present a case of a 15-year-old boy with keratitis-ichthyosis-deafness (KID) syndrome and DCS who experienced significant clinical response to treatment with adalimumab after failing multiple courses of oral antimicrobials, surgical interventions, intralesional corticosteroids, and topical therapies. Treatment with adalimumab led to reduction in drainage and pain and visible hair regrowth, highlighting its therapeutic potential in refractory, pediatric DCS including in the setting of syndromic skin disease.

#2

Treatment of Epidermal Pathology in a Pediatric Patient With Keratitis-Ichthyosis-Deafness (KID) Syndrome With Topical Mefenamic Acid.

Pediatric dermatology2026

Keratitis-ichthyosis-deafness (KID) syndrome is a rare genetic condition typically presenting at birth with ichthyosiform erythroderma and bilateral hearing loss and later progressing to diffuse keratodermatous plaques with scaling. The condition is associated with mutations in the GJB2 gene, which lead to aberrant activation of connexin hemichannels in keratinocytes. While no targeted treatment currently exists, a previously published in vivo study demonstrated that flufenamic acid (FFA), a nonspecific connexin inhibitor, reduces epidermal pathology in transgenic mouse models expressing the lethal GJB2 mutation. Herein, we report the case of a 5-year-old boy with KID syndrome presenting with painful, persistent scalp lesions, which responded remarkably well to topical mefenamic acid, offering a potential novel therapy for managing this challenging condition.

#3

An infant of KID syndrome along with Dandy-Walker malformation (DWM).

JAAD case reports2026 Jan
#4

Novel homozygous pathogenic AP1B1 variant in autosomal recessive keratitis-ichthyosis-deafness syndrome treated with acitretin.

BMJ case reports2025 Dec 25

We report a female in her early childhood with autosomal recessive keratitis-ichthyosis-deafness (KID) syndrome, presenting with congenital erythroderma, sensorineural deafness and developmental delays. Genetic analysis revealed a novel homozygous pathogenic variant in the AP1B1 gene. Initiation of acitretin therapy led to significant dermatologic improvement without adverse effects so far. This case underscores the rarity of autosomal recessive KID syndrome and highlights acitretin's potential as a therapeutic option.

#5

Virulence genes of pathogenic and clinical treatment in floppy kid syndrome.

Research in veterinary science2025 Aug

Floppy kid syndrome (FKS) is a metabolic disorder disease in newborn goats caused by intestinal dysbiosis. In this study, pathogens were isolated from the intestinal contents of FKS-affected goats (8 Escherichia coli isolates and 2 Staphylococcus aureus isoates), and virulence gene detection showed that Escherichia coli predominantly expressed HlyD (62.5 %) and K88 (37.5 %), with 37.5 % of isolates co-expressing HlyD/K88; all Staphylococcus aureus isolates carried the nuc, sea, sed, and FnbA. Antibiotic susceptibility testing revealed that amikacin exhibited the highest sensitivity against both pathogens, with sensitivity rates of 87.5 % in Escherichia coli and 100 % in Staphylococcus aureus. By combining virulence gene detection and antimicrobial susceptibility tests, a comprehensive treatment regimen (amikacin + sodium bicarbonate + glucose) was designed, and clinical trial indicated that the 3-day cure rate in the treatment group reached 100 % (24/24), significantly higher than 14.3 % (2/14) in the control group. This study revealed a synergistic pathogenic mechanism between Escherichia coli HlyD/K88 and Staphylococcus aureus enterotoxins, confirming that an amikacin-based combined treatment regimen can effectively alleviate FKS symptoms, thereby providing a theoretical basis for precision medication in FKS.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC126 artigos no totalmostrando 94

2026

Successful Use of Adalimumab for Dissecting Cellulitis in a Pediatric Patient With Keratitis-Ichthyosis-Deafness (KID) Syndrome.

Pediatric dermatology
2025

Case Report of Wound Treatment with Hyiodine Gel in an Occasional KID Syndrome Patient.

Journal of clinical medicine
2025

Novel homozygous pathogenic AP1B1 variant in autosomal recessive keratitis-ichthyosis-deafness syndrome treated with acitretin.

BMJ case reports
2026

An infant of KID syndrome along with Dandy-Walker malformation (DWM).

JAAD case reports
2025

Expanding the clinical spectrum: A case report of the first Jordanian presentation of KID syndrome with neurological and skeletal anomalies beyond the classical triad.

Medicine
2025

Cranial osteomyelitis in a patient with KID syndrome: Importance of thorough investigation in chronic wounds.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2025

Cochlear implantation in patients with keratitis-ichthyosis-deafness syndrome: A systematic review.

International journal of pediatric otorhinolaryngology
2025

Hypodontia in a child with keratitis-ichthyosis-deafness (KID) syndrome: a case report.

Journal of medical case reports
2025

KID syndrome: Laser-assisted daylight photodynamic therapy in the management of recalcitrant non-melanoma skin cancer.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2025

Goat-derived Lactobacillus amylovorus improved floppy kid syndrome via regulating gut microflora.

BMC veterinary research
2026

Treatment of Epidermal Pathology in a Pediatric Patient With Keratitis-Ichthyosis-Deafness (KID) Syndrome With Topical Mefenamic Acid.

Pediatric dermatology
2025

Ophthalmologic management in KID syndrome: Long-term clinical experience.

Archivos de la Sociedad Espanola de Oftalmologia
2025

Keratitis, Ichthyosis, and Deafness Syndrome with Endocarditis and Myelitis: A Rare Case Report.

Case reports in dermatology
2025

Genotype-Phenotype Correlations, Mortality, and Clinical Insights in Keratitis-Ichthyosis-Deafness Syndrome: A Comprehensive Review and Case Report.

American journal of medical genetics. Part A
2025

Assessing Hemichannel and Gap Junctional Channel Activities in HaCaT Keratinocyte Cells.

Methods in molecular biology (Clifton, N.J.)
2025

Virulence genes of pathogenic and clinical treatment in floppy kid syndrome.

Research in veterinary science
2025

Research in etiology of Floppy Kid Syndrome.

Frontiers in veterinary science
2025

Cochlear Implant Challenges in Children with Ichthyosis: A Systematic Review.

Genes
2025

Two cases of therapeutic scleral lenses for KID syndrome.

American journal of ophthalmology case reports
2024

Suspect dyskeratotic neoformations in a 7-year-old child with keratitis-ichthyosis-deafness syndrome: diagnostic, surgical and wound care management.

Dermatology reports
2025

Dental Abnormalities in Congenital Ichthyoses: Case Report and Review of the Literature.

Pediatric dermatology
2025

Connexin Hemichannel Inhibition and Human Genodermatoses.

The Journal of investigative dermatology
2024

Hyperkeratotic Nodule on the Knee in a Patient With KID Syndrome.

Cutis
2022

Use of Systemic Mycophenolate Mofetil Therapy in Ocular Surface Inflammatory Pathologies at the Initiative and Responsibility of the Ophthalmologist.

Middle East African journal of ophthalmology
2024

Emergence of multiple revertant keratinocyte clones in a patient with KID syndrome.

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

Ocular phenotype and therapeutic interventions in keratitis-ichthyosis-deafness (KID) syndrome.

Ophthalmic genetics
2023

Otological problems in ichthyosis: A literature review.

International journal of pediatric otorhinolaryngology
2023

Oral Nodular Chronic Hyperplastic Candidiasis of the Tongue: A Case Report.

Cureus
2023

Keratitis-ichthyosis-deafness syndrome and hidradenitis suppurativa.

JAAD case reports
2022

Keratitis-ichthyosis-deafness Syndrome with Heterozygous p.D50N in the GJB2 Gene in Two Serbian Adult Patients.

Balkan journal of medical genetics : BJMG
2023

Antibody gene transfer treatment drastically improves epidermal pathology in a keratitis ichthyosis deafness syndrome model using male mice.

EBioMedicine
2022

Expression of KID syndromic mutation Cx26S17F produces hyperactive hemichannels in supporting cells of the organ of Corti.

Frontiers in cell and developmental biology
2023

Keratitis-ichthyosis-deafness syndrome with lethal p.Ala88Val variant and severe hypercalcemia.

American journal of medical genetics. Part A
2022

KID Syndrome: A Rare Congenital Ichthyosiform Disorder.

Indian dermatology online journal
2022

The Clinical Manifestation of p.Asp50Asn Heterozygous Mutation of GJB2 Gene in 3 Members of a Family Is Similar to That of Clouston Syndrome.

Annals of dermatology
2022

Conformational changes and CO2-induced channel gating in connexin26.

Structure (London, England : 1993)
2021

Connexin hemichannel inhibition ameliorates epidermal pathology in a mouse model of keratitis ichthyosis deafness syndrome.

Scientific reports
2022

Parental mosaic cutaneous-gonadal GJB2 mutation: From epidermal nevus to inherited ichthyosis-deafness syndrome.

The Journal of dermatology
2021

Genome Sequences of Two Pseudomonas aeruginosa Isolates with Defects in Type III Secretion System Gene Expression from a Chronic Ankle Wound Infection.

Microbiology spectrum
2021

KID syndrome in a Malaysian child with identification of novel heterozygous missense mutation GJB2 c.581T>A(p. 194Phe>Tyr).

International journal of dermatology
2021

KID Syndrome and Hidradenitis Suppurativa: A Rare Association Responding to Surgical Treatment.

Skin appendage disorders
2021

Inherited ichthyosis and fungal infection: an update on pathogenesis and treatment strategies.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2021

Palmoplantar keratoderma with deafness phenotypic variability in a patient with an inherited GJB2 frameshift variant and novel missense variant.

Molecular genetics &amp; genomic medicine
2020

Novel Variant c.148G>T of GJB2 Gene in a 5-Year-Old Child with KID Syndrome.

Indian dermatology online journal
2020

Keratitis-ichthyosis-deafness syndrome: Phenotypic heterogeneity and treatment perspective of patients with p.Asp50Asn GJB2 mutation.

Dermatologic therapy
2020

Progressive Deformity of the Lower Limbs in a Patient with KID (Keratitis-Ichthyosis-Deafness) Syndrome.

Case reports in orthopedics
2020

Mulitmodal Corneal Imaging of Genetically Confirmed Keratitis-Ichthyosis-Deafness Syndrome.

Cornea
2020

Clinical, etiopathogenic, and therapeutic aspects of KID syndrome.

Dermatologic therapy
2020

KID Syndrome: A Rare Genodermatosis.

Indian dermatology online journal
2020

Verruciform xanthoma in recessive dystrophic epidermolysis bullosa and keratitis-ichthyosis-deafness syndrome: Report of two cases and a review of the literature.

Pediatric dermatology
2020

Allele-Specific Small Interfering RNA Corrects Aberrant Cellular Phenotype in Keratitis-Ichthyosis-Deafness Syndrome Keratinocytes.

The Journal of investigative dermatology
2019

A Rare Case of KID Syndrome: Erratum.

Advances in skin &amp; wound care
2019

Cx26 keratitis ichthyosis deafness syndrome mutations trigger alternative splicing of Cx26 to prevent expression and cause toxicity in vitro.

Royal Society open science
2019

A Rare Case of KID Syndrome: The Use of Hydrosurgery and Strategies for Antiseptic Wound Care.

Advances in skin &amp; wound care
2019

[Screening for ocular involvement in deaf children].

The Pan African medical journal
2019

Oral manifestations of KID syndrome: rare clinical case.

Stomatologiia
2020

Antenatal Findings of Keratitis-Ichthyosis-Deafness Syndrome.

Journal of obstetrics and gynaecology Canada : JOGC = Journal d'obstetrique et gynecologie du Canada : JOGC
2019

[Recurrent corneal ulcers as presenting sign of KID syndrome in a 68-year-old man].

Journal francais d'ophtalmologie
2019

Keratitis-Ichthyosis-Deafness Syndrome: Early Death Caused by the GJB2 Mutation p.Gly12Arg.

Acta dermato-venereologica
2019

A Tunisian family with a novel mutation in the gene CYP4F22 for lamellar ichthyosis and co-occurrence of hearing loss in a child due to mutation in the SLC26A4 gene.

International journal of dermatology
2019

The connexin26 human mutation N14K disrupts cytosolic intersubunit interactions and promotes channel opening.

The Journal of general physiology
2019

Ocular Surface Stem Cell Transplantation for Treatment of Keratitis-Ichthyosis-Deafness Syndrome.

Cornea
2018

Exome sequencing in a Chinese family reveals TTC9 mutation associated with keratitis-ichthyosis-deafness (KID) syndrome.

European journal of dermatology : EJD
2019

More than keratitis, ichthyosis, and deafness: Multisystem effects of lethal GJB2 mutations.

Journal of the American Academy of Dermatology
2018

Cross-sectional survey on disease severity in Japanese patients with harlequin ichthyosis/ichthyosis: Syndromic forms and quality-of-life analysis in a subgroup.

Journal of dermatological science
2018

Roles of aberrant hemichannel activities due to mutant connexin26 in the pathogenesis of KID syndrome.

Scientific reports
2018

Successfully Improving Visual Acuity in Keratitis-Ichthyosis-Deafness Syndrome Utilizing Gas-Permeable Lenses: A Case Report.

Eye &amp; contact lens
2018

Two Cases of Chronic Candidiasis in Keratitis-Ichthyosis-Deafness Syndrome.

The American Journal of dermatopathology
2018

The syndromic deafness mutation G12R impairs fast and slow gating in Cx26 hemichannels.

The Journal of general physiology
2017

Porokeratotic eccrine and hair follicle nevus: a report of two cases and review of the literature.

Anais brasileiros de dermatologia
2017

Visual impairment reversal with oral acitretin therapy in keratitis-ichthyosis-deafness (KID) syndrome.

JAAD case reports
2017

Cochlear Implantation in Patients with Keratitis-Ichthyosis-Deafness Syndrome: A Report of Two Cases.

Case reports in otolaryngology
2018

Severe Phenotype of Keratitis-Ichthyosis-Deafness Syndrome With Presumed Ocular Surface Squamous Neoplasia.

Cornea
2017

[Mutation analysis for a pedigree affected with keratitis-ichthyosis-deafness syndrome].

Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics
2017

Keratitis-ichthyosis-deafness syndrome accompanied by disseminated cutaneous fungal infection.

The Journal of dermatology
2019

Infectious keratitis in a patient with KID syndrome.

Enfermedades infecciosas y microbiologia clinica (English ed.)
2017

Systemic allergic contact dermatitis caused by methyl aminolaevulinate in a patient with keratosis-ichthyosis-deafness syndrome.

Contact dermatitis
2017

Revertant mosaicism repairs skin lesions in a patient with keratitis-ichthyosis-deafness syndrome by second-site mutations in connexin 26.

Human molecular genetics
2017

Vegetating Candidiasis: A Mimicker of Squamous Cell Carcinoma in Keratitis Ichthyosis Deafness Syndrome.

Pediatric dermatology
2016

Altered CO2 sensitivity of connexin26 mutant hemichannels in vitro.

Physiological reports
2016

Comparative functional characterization of novel non-syndromic GJB2 gene variant p.Gly45Arg and lethal syndromic variant p.Gly45Glu.

PeerJ
2016

Diminution of Langerhans cells in keratitis, ichthyosis and deafness (KID) syndrome patient with recalcitrant cutaneous candidiasis.

Journal of the European Academy of Dermatology and Venereology : JEADV
2016

Successful treatment of pityriasis lichenoides chronica with narrow-band ultraviolet B therapy in a patient with Keratitis-Ichthyosis-Deafness syndrome: a case report.

Dermatology online journal
2016

Connexin 26 (GJB2) mutation in an Argentinean patient with keratitis-ichthyosis-deafness (KID) syndrome: a case report.

BMC medical genetics
2016

Inherited ichthyosis: Syndromic forms.

The Journal of dermatology
2016

Keratoprosthesis in pediatric keratitis-icthyosiform-deafness syndrome.

Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus
2016

Altered cellular localization and hemichannel activities of KID syndrome associated connexin26 I30N and D50Y mutations.

BMC cell biology
2016

Phenotype in a patient with p.D50N mutation in GJB2 gene resemble both KID and Clouston syndromes.

International journal of pediatric otorhinolaryngology
2016

From Hyperactive Connexin26 Hemichannels to Impairments in Epidermal Calcium Gradient and Permeability Barrier in the Keratitis-Ichthyosis-Deafness Syndrome.

The Journal of investigative dermatology
2015

Connexin hemichannels influence genetically determined inflammatory and hyperproliferative skin diseases.

Pharmacological research
2015

Altered epidermal lipid processing and calcium distribution in the KID syndrome mouse model Cx26S17F.

FEBS letters
2015

Oral squamous cell carcinoma in a patient with keratitis-ichthyosis-deafness syndrome: a rare case.

Oral surgery, oral medicine, oral pathology and oral radiology
2015

Keratitis-ichthyosis-deafness syndrome-associated Cx26 mutants produce nonfunctional gap junctions but hyperactive hemichannels when co-expressed with wild type Cx43.

The Journal of investigative dermatology
2015

Phenotypic variability in gap junction syndromic skin disorders: experience from KID and Clouston syndromes' clinical diagnostics.

Journal of applied genetics
Ver todos os 126 no EuropePMC

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

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Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

Ainda não existe comunidade no Raras para Síndrome KID

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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. Successful Use of Adalimumab for Dissecting Cellulitis in a Pediatric Patient With Keratitis-Ichthyosis-Deafness (KID) Syndrome.
    Pediatric dermatology· 2026· PMID 41663077mais citado
  2. Treatment of Epidermal Pathology in a Pediatric Patient With Keratitis-Ichthyosis-Deafness (KID) Syndrome With&#xa0;Topical Mefenamic Acid.
    Pediatric dermatology· 2026· PMID 40814260mais citado
  3. An infant of KID syndrome along with Dandy-Walker malformation (DWM).
    JAAD case reports· 2026· PMID 41446690mais citado
  4. Novel homozygous pathogenic AP1B1 variant in autosomal recessive keratitis-ichthyosis-deafness syndrome treated with acitretin.
    BMJ case reports· 2025· PMID 41453769mais citado
  5. Virulence genes of pathogenic and clinical treatment in floppy kid syndrome.
    Research in veterinary science· 2025· PMID 40413826mais citado
  6. Macrophage Extracellular Vesicles: Therapeutic Strategies for Corneal Fibrosis in Rare Diseases.
    Biomolecules· 2026· PMID 41897284recente
  7. Case Report of Wound Treatment with Hyiodine Gel in an Occasional KID Syndrome Patient.
    J Clin Med· 2025· PMID 41517259recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:477(Orphanet)
  2. MONDO:0018781(MONDO)
  3. GARD:3113(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. Q1345746(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

Síndrome KID
Compêndio · Raras BR

Síndrome KID

ORPHA:477 · MONDO:0018781
Prevalência
<1 / 1 000 000
Casos
100 casos conhecidos
Herança
Autosomal dominant, Autosomal recessive, Not applicable
CID-10
Q80.8 · Outras ictioses congênitas
CID-11
Início
Neonatal
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C1275089
EuropePMC
Wikidata
Papers 10a
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