Introdução
O que você precisa saber de cara
Nevo epidérmico verrucoso linear inflamatório (ILVEN) é um distúrbio cutâneo incomum caracterizado por placas pruriginosas (com coceira), avermelhadas (eritêmatosas) e elevadas (hiperceratóticas) que seguem as linhas de desenvolvimento das células da pele. A erupção cutânea geralmente se manifesta na infância ou primeira infância, com predominância feminina e afeta mais frequentemente os membros inferiores. O ILVEN é causado por mutações somáticas durante o estágio embrionário que resultam em mosaicismo genético visível. Não há cura, mas diferentes tratamentos médicos podem aliviar os sintomas.
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
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)
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Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Pesquisa e ensaios clínicos
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Publicações mais relevantes
Linear psoriasis and Koebner phenomenon: A review.
Linear psoriasis (LP) is an unusual form of psoriasis with an unidentified prevalence and is characterized by psoriatic papules and plaques in a Blaschko linear distribution. Based on clinical features, this disorder is divided into two types: isolated LP and superimposed LP. Previous studies have suggested that LP presenting linear appearances is closely related to the Kobner phenomenon (KP) caused by external provocation, including trauma, skin incision, drugs, infections, and striae distensae. Pathogenesis of LP is mainly attributed to the concept of genetic mosaicism but not completely illustrated until now. In this review, we summarize its epidemiological characteristics, clinicopathological features, diagnosis/differential diagnosis, corresponding therapies, focus on the possible pathogenesis of LP, and explore the relationship between LP and KP.
Hypopigmented lesions admixed with verrucous papules in a child with epidermolytic verrucous epidermal nevus.
A female infant presented with asymptomatic hyperpigmented and hypopigmented verrucous papules and plaques along Blaschko's lines on the right side of her body, with an absence of blistering, flexural accentuation or family history. A systemic examination was normal. Differentials considered were epidermal nevus, incontinentia pigmenti stage 2, lichen striatus, inflammatory linear verrucous epidermal nevus and linear epidermolytic ichthyosis (EI). A skin biopsy revealed epidermolytic hyperkeratosis (EHK). Mutation analysis could not be done as the parents were unwilling. EHK is classically seen in EI, which presents with marked blistering that resolves within a few months with hyperkeratosis; there is also accentuation around the flexures. Mosaic epidermolytic ichthyosis (mEI), a rare variant, is localised or along Blaschko's lines, as in our patient. It differs from classic EI with the absence of blistering, flexural involvement and erythroderma.Epidermolytic verrucous epidermal nevus (VEN) and mEI refer to the same condition. Approximately 5% of all cases of VEN show the histological finding of EHK. Mosaic EI represents a somatic mutation of the KRT1 and/or KRT10 genes. Evaluation for this is important as mosaic conditions may lead to full-blown EI in the offspring of affected individuals.This case is being reported due to the unusual presentation of mEI as hypopigmented with hyperpigmented lesions and also to stress the importance of biopsy when a child presents with blaschkoid lesions.
Linear psoriasis or inflammatory linear verrucous epidermal nevus? A pediatric case unraveling the mosaic puzzle.
Linear psoriasiform eruptions in children are diagnostically challenging, particularly when they follow Blaschko's lines. The main differential diagnosis includes inflammatory linear verrucous epidermal nevus (ILVEN) and linear psoriasis (LP), a rare mosaic variant. We describe the case of a 5-year-old boy with extensive unilateral lesions clinically suggestive of both entities. Histology revealed Munro microabscesses and psoriasiform hyperplasia. The patient showed rapid improvement with topical calcipotriol/betamethasone, followed by long-term remission. Notably, after 8 years of follow-up, the lesions remained strictly confined to the original Blaschkoid distribution, with no extracutaneous involvement and only mild recurrences. This clinical course strongly supports the diagnosis of isolated linear psoriasis (type 1 mosaicism). Few pediatric cases in the literature have documented such a long and stable evolution, making this report a valuable contribution to the distinction between LP and ILVEN. The case highlights the importance of integrating clinical, histological, and longitudinal data when evaluating linear dermatoses in children.
Exploring the Application of Secukinumab in Patients With Linear Psoriasis and Inflammatory Linear Verrucous Epidermal Nevus: A Case Study and Mini-Review.
Postzygotic Gain-of-Function Variant in TRPM4 and Inflammatory Linear Verrucous Epidermal Nevus.
This case report describes a young woman with diffuse pruritic erythematous plaques and papules since birth who presented with coalesced spiked keratotic papules on the palms and chalky white fingernails.
Publicações recentes
Inflammatory Linear Verrucous Epidermal Nevus Refractory to IL-17A Blockade but Responsive to Tofacitinib.
Linear psoriasis and Koebner phenomenon: A review.
Hypopigmented lesions admixed with verrucous papules in a child with epidermolytic verrucous epidermal nevus.
Topical Treatments for Rare Genetic Dermatological Diseases: A Narrative Review.
Epidermal nevus: a rare presentation of congenital epidermal hamartoma in a young female.
📚 EuropePMC114 artigos no totalmostrando 61
Linear psoriasis and Koebner phenomenon: A review.
Journal of research in medical sciences : the official journal of Isfahan University of Medical SciencesHypopigmented lesions admixed with verrucous papules in a child with epidermolytic verrucous epidermal nevus.
BMJ case reportsTopical Treatments for Rare Genetic Dermatological Diseases: A Narrative Review.
Pharmaceuticals (Basel, Switzerland)Epidermal nevus: a rare presentation of congenital epidermal hamartoma in a young female.
The Pan African medical journalTemporary improvement of porokeratotic inflammatory linear verrucous epidermal nevus with fractionated CO2 laser resurfacing.
Dermatology online journalExploring the Application of Secukinumab in Patients With Linear Psoriasis and Inflammatory Linear Verrucous Epidermal Nevus: A Case Study and Mini-Review.
International journal of dermatologyPostzygotic Gain-of-Function Variant in TRPM4 and Inflammatory Linear Verrucous Epidermal Nevus.
JAMA dermatologyAn unusual case of inflammatory linear verrucous epidermal nevus on the labia majora.
Indian journal of sexually transmitted diseases and AIDSLinear psoriasis or inflammatory linear verrucous epidermal nevus? A pediatric case unraveling the mosaic puzzle.
Dermatology reportsInflammatory Linear Verrucous Epidermal Nevus.
Journal of cutaneous medicine and surgeryLinear erythematous papulovesicular rash in a newborn.
JAAD case reportsInflammatory Linear Verrucous Epidermal Nevus (ILVEN): A Scarce Cognate with a Neoplasia.
Indian journal of surgical oncologyInflammatory linear verrucous epidermal nevus successfully treated with ustekinumab.
JAAD case reportsUnilateral widespread inflammatory linear verrucous epidermal nevus with ipsilateral limb contracture along the lines of Blaschko: A case report.
The Journal of international medical researchSuccessful Treatment of Linear Psoriasis With the IL-17a-Antagonist Ixekizumab: A Case Report.
Psoriasis (Auckland, N.Z.)Cost-utility and actionability of genomic sequencing for inflammatory linear verrucous epidermal nevus.
Journal of the American Academy of DermatologySecukinumab treatment for inflammatory linear verrucous epidermal nevus.
The Journal of dermatologyInflammatory linear verrucous epidermal nevus: A paradigm of progress.
Journal of the American Academy of DermatologyAdult-onset inflammatory linear verrucous epidermal nevus successfully treated with intralesional steroid.
JAAD case reportsInflammatory linear verrucous epidermal nevus should be genotyped to direct treatment and genetic counseling.
Journal of the American Academy of DermatologyInflammatory linear verrucous epidermal nevus in groin and labia with claudication: Successfully treated with photodynamic therapy.
Photodiagnosis and photodynamic therapyGiant inflammatory linear verrucous epidermal nevus: difficulties in the management.
Postepy dermatologii i alergologiiInflammatory Linear Verrucous Epidermal Nevus Assessment With in Vivo Reflectance Confocal Microscopy: an Easy Option to Consider.
Dermatology practical & conceptualA Case of Naevoid Blaschkoid Psoriasis in a Two-Year-Old Child.
Indian journal of dermatologyInflammatory linear verrucous epidermal nevus-A rare variant of epidermal nevus.
SemergenInflammatory linear verrucous epidermal nevus (ILVEN) encompasses a spectrum of inflammatory mosaic disorders.
Pediatric dermatologyA case of inflammatory linear verrucous epidermal nevus successfully treated with Brodalumab.
Pediatric dermatologyInflammatory linear verrucous epidermal nevus in a patient with diabetes.
The Pan African medical journalDifferences in clinical characteristics and lesion proteomics between inflammatory linear verrucous epidermal nevus and local verrucous epidermal nevus.
Journal of proteomics[Benign skin neoplasms in children].
Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte GebieteDermatoscopic and confocal microscopy features of widespread inflammatory linear verrucous epidermal nevus.
Italian journal of dermatology and venereologySuccessful Amelioration of Inflammatory Linear Verrucous Epidermal Nevus with Topical Sirolimus.
Annals of dermatologyPsoriasis over healed inflammatory linear verrucous epidermal nevus: Wolf´s isotopic response.
The Australasian journal of dermatologyAdult-onset inflammatory linear verrucous epidermal nevus: Immunohistochemical studies and review of the literature.
Journal of cutaneous pathologyLinear crusted papules in an infant.
JAAD case reportsOff-Label Therapeutic Potential of Crisaborole.
Journal of cutaneous medicine and surgeryNail lichen striatus: Is dermoscopy useful for the diagnosis?
Pediatric dermatologyInflammatory linear verrucous epidermal nevus treated successfully with crisaborole ointment in a 5-year-old boy.
Pediatric dermatologySuccessful treatment of inflammatory linear verrucous epidermal nevus (ILVEN) with 308-nm excimer laser: Patient patient required.
Photodermatology, photoimmunology & photomedicineConnexin43 mutations linked to skin disease have augmented hemichannel activity.
Scientific reportsGenetic variant in the NSDHL gene in a cat with multiple congenital lesions resembling inflammatory linear verrucous epidermal nevi.
Veterinary dermatologyVerruciform xanthoma overlying inflammatory linear verrucous epidermal nevus and in broad segmental distribution.
BMJ case reportsInflammatory linear verrucous epidermal nevus responsive to 308-nm excimer laser treatment.
CutisTreatment of inflammatory linear verrucous epidermal nevus pruritus with thalidomide.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGWidely Spread Unilateral Inflammatory Linear Verrucous Epidermal Nevus (ILVEN).
Case reports in dermatologyInflammatory linear verrucous epidermal nevus associated with erosive monoarthritis.
Pediatric dermatologyReconstruction of a buttock defect after excision of inflammatory linear verrucous epidermal nevus.
Dermatologic therapySuccessful Treatment of Inflammatory Linear Verrucous Epidermal Nevus with Concomitant Psoriasis Using Etanercept.
Case reports in dermatologyThe Successful Treatment of a Case of Linear Psoriasis with Ixekizumab.
Case reports in dermatological medicineIs Ki-67, keratin 16, involucrin, and filaggrin immunostaining sufficient to diagnose inflammatory linear verrucous epidermal nevus? A report of eight cases and a comparison with psoriasis vulgaris.
Anais brasileiros de dermatologiaEpidermal nevus syndromes: New insights into whorls and swirls.
Pediatric dermatologyDermoscopy of linear dermatosis along Blaschko's line in childhood: Lichen striatus versus inflammatory linear verrucous epidermal nevus.
The Journal of dermatologyA Case of "Inflammatory Linear Verrucous Epidermal Nevus" (ILVEN) Treated with CO2 Laser Ablation.
Open access Macedonian journal of medical sciencesILVEN - COMPLETE REMISSION AFTER ADMINISTRATION OF TOPICAL CORTICOSTEROID (CASE REVIEW).
Georgian medical newsInflammatory Linear Verrucous Epidermal Nevus with a Postzygotic GJA1 Mutation Is a Mosaic Erythrokeratodermia Variabilis et Progressiva.
The Journal of investigative dermatologyInflammatory linear verrucous epidermal nevus and regional odontodysplasia: A rare sorority.
Indian journal of dentistryGenital/Perigenital Inflammatory Linear Verrucous Epidermal Nevus: A Case Series.
Indian journal of dermatologyLinear Darier's disease: A case with bilateral presentation.
Indian dermatology online journalInflammatory linear verrucous epidermal nevus: an unusual manifestation.
Giornale italiano di dermatologia e venereologia : organo ufficiale, Societa italiana di dermatologia e sifilografiaDifferential Diagnosis of Linear Eruptions in Children.
Pediatric annalsA Case of Inflammatory Linear Verrucous Epidermal Nevus on the Upper Eyelid.
Indian journal of dermatologyAssociações
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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.
- Linear psoriasis and Koebner phenomenon: A review.Journal of research in medical sciences : the official journal of Isfahan University of Medical Sciences· 2026· PMID 41852788mais citado
- Hypopigmented lesions admixed with verrucous papules in a child with epidermolytic verrucous epidermal nevus.
- Linear psoriasis or inflammatory linear verrucous epidermal nevus? A pediatric case unraveling the mosaic puzzle.
- Exploring the Application of Secukinumab in Patients With Linear Psoriasis and Inflammatory Linear Verrucous Epidermal Nevus: A Case Study and Mini-Review.
- Postzygotic Gain-of-Function Variant in TRPM4 and Inflammatory Linear Verrucous Epidermal Nevus.
- Inflammatory Linear Verrucous Epidermal Nevus Refractory to IL-17A Blockade but Responsive to Tofacitinib.
- Topical Treatments for Rare Genetic Dermatological Diseases: A Narrative Review.
- Epidermal nevus: a rare presentation of congenital epidermal hamartoma in a young female.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:79466(Orphanet)
- MONDO:0019318(MONDO)
- GARD:5484(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q6030093(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
