É uma doença autoimune caracterizada pela formação de bolhas abaixo da epiderme e pelo depósito linear de autoanticorpos na união entre a derme e a epiderme. Os autoanticorpos acumulados são do tipo imunoglobulina A (IgA) e, ocasionalmente, imunoglobulina G (IgG), e atacam as proteínas da membrana que serve de base para a epiderme. Essa condição, às vezes, está associada a alguns tipos de câncer e ao uso de certos medicamentos (como, por exemplo, a vancomicina).
Introdução
O que você precisa saber de cara
A dermatose por acúmulo linear de IgA é uma doença autoimune rara em que o próprio sistema de defesa deposita anticorpos na pele, formando bolhas e pequenas feridas avermelhadas. Ela pode se manifestar em pessoas de qualquer idade e, além das bolhas na pele que costumam coçar, pode provocar feridas na boca e sangramentos no nariz. O quadro pode surgir sem motivo aparente ou estar associado ao uso de certos medicamentos, como a vancomicina. Esta doença não possui um protocolo clínico com medicamentos fornecidos em listas específicas pelo SUS, e as pesquisas científicas mencionam remédios como dapsona e corticoides em estudos internacionais.
É uma doença autoimune caracterizada pela formação de bolhas abaixo da epiderme e pelo depósito linear de autoanticorpos na união entre a derme e a epiderme. Os autoanticorpos acumulados são do tipo imunoglobulina A (IgA) e, ocasionalmente, imunoglobulina G (IgG), e atacam as proteínas da membrana que serve de base para a epiderme. Essa condição, às vezes, está associada a alguns tipos de câncer e ao uso de certos medicamentos (como, por exemplo, a vancomicina).
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
Encontrou um erro ou informação desatualizada? Sugira uma correção →
Entender a doença
Do básico ao detalhe, leia no seu ritmo
Preparando trilha educativa...
Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 6 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 11 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
Encontrou um erro ou informação desatualizada? Sugira uma correção →
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)
🇧🇷 Atendimento SUS — Dermatose por acúmulo linear de IgA
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
Ensaios em destaque
Nenhum país com ensaio aberto no momento.
Pesquisa e ensaios clínicos
1 ensaios clínicos encontrados.
Publicações mais relevantes
European S2k guidelines on management of autoimmune blistering diseases in children and adolescents.
Autoimmune blistering diseases (AIBDs) in children and adolescents present a major challenge in diagnosis and management because of their rarity and the presence of other differential diagnoses with blistering. They are potentially serious, and timely diagnosis and optimal management can help prevent morbidity related to both the diseases and their treatments. The Autoimmune Blistering Diseases Task Force of the European Academy of Dermatology and Venereology (EADV) has initiated evidence-based, expert-informed clinical guidelines for the diagnosis, treatment and monitoring of paediatric AIBDs. A writing group conducted a systematic review of the available literature on AIBDs in children and adolescents, and since there is limited strong evidence in the literature, recommendations were formulated by consensus over three rounds of structured feedback and revision by the Core Group and Expert Group of the task force (32 voting experts; >75% agreement threshold). Although all AIBDs-including intra-epidermal and subepidermal blistering disorders-are reported in children, linear IgA dermatosis (LAD) is the most common (42-83%). The diagnostic approach in the paediatric age group does not differ from that in adults. Treatment recommendations are tailored to paediatric patients, taking into account developmental pharmacokinetics, potential adverse effects and the impact of disease and therapy on quality of life. These guidelines provide the first comprehensive, paediatric-focused framework for the diagnosis and management of AIBDs. Implementing the recommendations should harmonize care, reduce treatment-related toxicity and improve quality of life for affected children while steering future research towards critical knowledge gaps.
A Rare Type of Bullous Disorder in a Child. Can It Be Chronic Bullous Disease of Childhood?
Chronic bullous disease of childhood (CBDC), also known as childhood linear IgA bullous dermatosis, is a rare autoimmune disorder characterized by sub-epidermal blistering and linear deposition of immunoglobulin A at the demo-epidermal junction. Clinical manifestations in children include tense bulla (arranged annularly in the form of ring of pearl) and vesicles over the erythematous skin. Diagnosis is confirmed by skin biopsy from lesions showing sub-epidermal blisters in histopathological examination and linear deposition of IgA at the dermal-epidermal junction in direct immunofluorescence. Here, we report a case of CBDC in a 5-year-old child who was treated successfully with oral dapsone showing improvement in the skin lesions.
Annular lesions in childhood: A narrative review.
Annular skin lesions are ring-shaped manifestations characterized by an erythematous margin and a preserved or atrophic center. Their pathophysiology involves mechanisms such as peripheral inflammatory mediator expansion or immune tolerance at the center of the lesions. This article reviews some of the most common annular lesions observed in pediatric populations within our setting. We included urticarial vasculitis, childhood cutaneous lupus erythematous, Still's disease, annular granuloma, centriphugal annular erythema, pigmentary dermatoses, erythema migrans, multiform urticaria, acute hemorrhagic edema of the infancy, infectious dermatoses such as dermatophyte infections, and blistering conditions like linear IgA dermatosis, highlighting their clinical presentations, diagnostic approaches, and treatment options, emphasizing the importance of clinical correlation, biopsy findings, and appropriate therapeutic strategies. This review will assist clinicians in diagnosing diseases associated with annular skin lesions.
Refractory linear IgA dermatosis in childhood: a successful response to rituximab.
Ramipril-Induced Annular Bullous Pemphigoid: A Clinical Mimic of Linear IgA Dermatosis.
Publicações recentes
Refractory linear IgA dermatosis in childhood: a successful response to rituximab.
European S2k guidelines on management of autoimmune blistering diseases in children and adolescents.
Ramipril-Induced Annular Bullous Pemphigoid: A Clinical Mimic of Linear IgA Dermatosis.
Idiopathic linear IgA dermatosis.
A Rare Type of Bullous Disorder in a Child. Can It Be Chronic Bullous Disease of Childhood?
📚 EuropePMC97 artigos no totalmostrando 51
Refractory linear IgA dermatosis in childhood: a successful response to rituximab.
Anais brasileiros de dermatologiaEuropean S2k guidelines on management of autoimmune blistering diseases in children and adolescents.
Journal of the European Academy of Dermatology and Venereology : JEADVRamipril-Induced Annular Bullous Pemphigoid: A Clinical Mimic of Linear IgA Dermatosis.
The Eurasian journal of medicineIdiopathic linear IgA dermatosis.
Medicina clinicaA Rare Type of Bullous Disorder in a Child. Can It Be Chronic Bullous Disease of Childhood?
Clinical case reportsRisk of persistent hypogammaglobulinaemia in children with autoimmune bullous dermatoses treated with rituximab.
Journal of the European Academy of Dermatology and Venereology : JEADVAnnular lesions in childhood: A narrative review.
Archives de pediatrie : organe officiel de la Societe francaise de pediatrieLevamisole associated with linear IgA dermatosis.
Clinical toxicology (Philadelphia, Pa.)Childhood Linear IgA Dermatosis Successfully Treated with the Combination of Dapsone and Sulfasalazine.
Case reports in dermatologyAtypical bullous systemic lupus erythematosus with features of linear IgA bullous dermatosis.
JAAD case reportsAzathioprine Hypersensitivity Syndrome Mimicking Herpes Zoster and Linear IgA Dermatosis Presentation: A Case Report.
Acta dermato-venereologicaState-of-the-art diagnosis of autoimmune blistering diseases.
Frontiers in immunologyS2k guidelines on diagnosis and treatment of linear IgA dermatosis initiated by the European Academy of Dermatology and Venereology.
Journal of the European Academy of Dermatology and Venereology : JEADVLinear IgA dermatosis secondary to treatment with piperacillin-tazobactam in a nonagenarian patient.
Postepy dermatologii i alergologiiImmunohistochemical Evaluation of C4d and C3d Markers in Bullous Pemphigoid as a Substitute for Direct Immunofluorescence Technique.
Indian journal of dermatologyRetrospective analysis of autoimmune bullous diseases in Middle Franconia.
Frontiers in immunologyGuideline for allergological diagnosis of drug hypersensitivity reactions: S2k Guideline of the German Society for Allergology and Clinical Immunology (DGAKI) in cooperation with the German Dermatological Society (DDG), the Association of German Allergologists (ÄDA), the German Society for Pediatric Allergology (GPA), the German Contact Dermatitis Research Group (DKG), the German Society for Pneumology (DGP), the German Society of Otorhinolaryngology, Head and Neck Surgery, the Austrian Society of Allergology and Immunology (ÖGAI), the Austrian Society of Dermatology and Venereology (ÖGDV), the German Academy of Allergology and Environmental Medicine (DAAU), and the German Documentation Center for Severe Skin Reactions (dZh).
Allergologie selectStevens-johnson Syndrome and Toxic Epidermal Necrolysis: An Overview of Diagnosis, Therapy Options and Prognosis of Patients.
Recent advances in inflammation & allergy drug discoveryManagement Options for Linear Immunoglobulin A (IgA) Bullous Dermatosis: A Literature Review.
CureusSpontaneous autoimmune subepidermal blistering diseases in animals: a comprehensive review.
BMC veterinary researchEpidermolysis Bullosa Acquisita-Current and Emerging Treatments.
Journal of clinical medicineType XVII collagen: Relevance of distinct epitopes, complement-independent effects, and association with neurological disorders in pemphigoid disorders.
Frontiers in immunologyMorphological Spectrum of Vesiculobullous Skin Lesions: An Institutional Perspective.
CureusDiffuse cutaneous mastocytosis masquerading as linear IgA bullous dermatosis of childhood.
Dermatology reportsNorfloxacin-Induced Linear IgA Dermatosis.
SkinmedIntravenous Immunoglobulins in a Series of 32 Rare and Recalcitrant Immune Dermatoses.
Acta dermato-venereologicaBullous lesions in a young adolescent postrenal transplant.
Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi ArabiaLinear IgA dermatosis of the childhood-Report of an amoxicillin-induced case.
Dermatologic therapyLinear IgA dermatosis in association with angioimmunoblastic T-cell lymphoma infiltrating the skin: A case report with literature review.
Journal of cutaneous pathologyLichen Planus Pemphigoides: From Lichenoid Inflammation to Autoantibody-Mediated Blistering.
Frontiers in immunologyCephalosporin-induced linear IgA dermatosis in a child: Case report and literature review.
Dermatologic therapyDrug-induced Linear IgA Bullous Dermatosis: A Case Report and Review of the Literature.
Acta dermato-venereologica["Decoratively figured blisters" on the whole integument in initially diagnosed ulcerative colitis].
Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte GebietePreviously misdiagnosed linear IgA dermatosis resolved with dapsone.
BMJ case reportsBlistering diseases in the mature patient.
Clinics in dermatology[Humoral immune diseases: Cutaneous vasculitis and auto-immune bullous dermatoses].
Annales de pathologieLinear IgA dermatosis: An atypical manifestation of a rare disease.
Pediatrics and neonatologySpectrum of Autoimmune Bullous Diseases in Northern Greece. A 4-year Retrospective Study and Review of the Literature.
Acta dermatovenerologica Croatica : ADCC-Terminal Processing of Collagen XVII Induces Neoepitopes for Linear IgA Dermatosis Autoantibodies.
The Journal of investigative dermatologyLinear immunoglobulin A/G bullous dermatosis associated with ulcerative colitis.
The Journal of dermatologyLinear IgA dermatosis limited to the mucosal membranes as a rare cause of dysphagia.
Ear, nose, & throat journalVancomycin-associated linear IgA disease mimicking toxic epidermal necrolysis.
Anais brasileiros de dermatologiaLinear immunoglobulin A dermatosis mimicking toxic epidermal necrolysis: a case report of etanercept treatment.
The British journal of dermatologyLinear IgA dermatosis after infliximab infusion for ulcerative colitis.
JAAD case reportsEosinophilic pneumonia as complication of linear IgA dermatosis treated with dapsone.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGParaneoplastic Pemphigus and Autoimmune Blistering Diseases Associated with Neoplasm: Characteristics, Diagnosis, Associated Neoplasms, Proposed Pathogenesis, Treatment.
American journal of clinical dermatology[Topical corticosteroids as a therapeutic alternative in linear immunoglobulin A bullous dermatosis in childhood: case report].
Archivos argentinos de pediatriaA case of linear IgA dermatosis successfully treated with tetracycline and niacinamide.
International journal of dermatologyGeneralized linear IgA dermatosis with palmar involvement.
Dermatology online journalLinear IgA dermatosis adult variant with oral manifestation: A rare case report.
Journal of oral and maxillofacial pathology : JOMFPConcurrent Drug-Induced Linear Immunoglobulin A Dermatosis and Immunoglobulin A Nephropathy.
Annals of dermatologyAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Dermatose por acúmulo linear de IgA.
É de uma associação que acompanha esta doença? Fale com a gente →
Doença com base genética
Um médico geneticista pode ajudar no diagnóstico de Dermatose por acúmulo linear de IgA e no aconselhamento genético da família.
Doenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
Perguntas frequentes
O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu
É uma doença autoimune rara da pele em que anticorpos, principalmente IgA, acumulam-se em linha na junção entre as camadas da pele. Isso resulta no surgimento de bolhas abaixo da epiderme e lesões cutâneas.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
6 publicações do grafo RarasNet (PubMed) · 5 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.
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.
Citar este verbete
Raras. (s.d.). Dermatose por acúmulo linear de IgA. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/dermatose-por-acumulo-linear-de-iga
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Dermatose por acúmulo linear de IgA
📋 Origem dos dados
Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.
- Doença rara (ontologia)
- fonte: Orphanet
- Identificador unificado
- fonte: MONDO
- Codificação WHO/SUS
- fonte: WHO ICD-10 / DATASUS
- CID-11 (futuro)
- fonte: WHO ICD-11
- NIH/GARD
- fonte: GARD (NIH)
- Indexação biomédica
- fonte: MeSH (NLM)
- Dado público estruturado
- fonte: Wikidata
- Reposicionamento
- fonte: Drug Repurposing Hub
