Linear IgA bullous dermatosis: report of an exuberant clinical case and literature review

Detalhes bibliográficos
Autor(a) principal: Pereira, Sandra
Data de Publicação: 2019
Outros Autores: Martins, Alexandra, Oliveira, Teresa, Monteiro, Virgínia
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: https://doi.org/10.25753/BirthGrowthMJ.v27.i4.13021
Resumo: Introduction: Linear IgA dermatosis (LIGAD) is a rare acquired disease, with a probable autoimmune origin. Its differential diagnosis involves other bullous dermatosis. Case Report: A previously healthy 12-month-old male was observed at the Emergency Department due to a 4-day itchy vesiculobullous rash in the perineal region, lower abdomen, hands, and feet. Analytical study was normal. Flucloxacillin and hydroxyzine were initiated without improvement. New (some of which confluent) lesions, erosions, and serohematic crusts developed on the periphery of previous lesions. A skin biopsy was performed at this time, revealing a subepidermal blister with neutrophilic infiltrate at histological examination. Direct immunofluorescence uncovered linear IgA deposits along the basement membrane. Lesion remission occurred without further therapeutic measures. Discussion: Although clinically exuberant, LIGAD is usually a self-limited disease. A high degree of suspicion is important, since immunofluorescence is diagnostic and pathognomonic, avoiding late diagnosis, unnecessary treatments, and parental anxiety.
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spelling Linear IgA bullous dermatosis: report of an exuberant clinical case and literature reviewDermatose bolhosa IgA Linear: relato de um caso exuberante e revisão da literaturaCase ReportsIntroduction: Linear IgA dermatosis (LIGAD) is a rare acquired disease, with a probable autoimmune origin. Its differential diagnosis involves other bullous dermatosis. Case Report: A previously healthy 12-month-old male was observed at the Emergency Department due to a 4-day itchy vesiculobullous rash in the perineal region, lower abdomen, hands, and feet. Analytical study was normal. Flucloxacillin and hydroxyzine were initiated without improvement. New (some of which confluent) lesions, erosions, and serohematic crusts developed on the periphery of previous lesions. A skin biopsy was performed at this time, revealing a subepidermal blister with neutrophilic infiltrate at histological examination. Direct immunofluorescence uncovered linear IgA deposits along the basement membrane. Lesion remission occurred without further therapeutic measures. Discussion: Although clinically exuberant, LIGAD is usually a self-limited disease. A high degree of suspicion is important, since immunofluorescence is diagnostic and pathognomonic, avoiding late diagnosis, unnecessary treatments, and parental anxiety.Introdução: A dermatose IgA linear (DIGAL) infantil é uma doença adquirida rara, de origem autoimune provável, que implica diagnóstico diferencial com outras dermatoses bolhosas. Caso Clínico: Uma criança de 12 meses, saudável, do género masculino, foi observada no Serviço de Urgência por erupção vesico-bolhosa, pruriginosa, com quatro dias de evolução, na região perineal, abdominal inferior, mãos e pés. O estudo analítico não revelou alterações. A criança iniciou flucloxacilina e hidroxizina, sem melhoria. Verificou-se o aparecimento de novas lesões (algumas confluentes) na periferia das antigas, assim como erosões e crostas serohemorrágicas. Foi efetuada uma biópsia cutânea, cujo exame histológico revelou bolha subepidérmica com infiltrado neutrofílico. A imunofluorescência direta revelou depósitos lineares de IgA ao longo da membrana basal. Observou-se evolução com remissão das lesões sem medidas terapêuticas adicionais. Conclusão: A DIGAL, apesar de clinicamente exuberante, é geralmente autolimitada. É necessário um elevado índice de suspeição, já que a imunofluorescência é diagnóstica e patognomónica, permitindo evitar diagnósticos tardios, tratamentos desnecessários e ansiedade parental.Centro Hospitalar Universitário do Porto2019-01-02T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articlehttps://doi.org/10.25753/BirthGrowthMJ.v27.i4.13021eng2183-9417Pereira, SandraMartins, AlexandraOliveira, TeresaMonteiro, Virgíniainfo:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2022-09-21T14:55:27Zoai:ojs.revistas.rcaap.pt:article/13021Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T15:56:25.375101Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse
dc.title.none.fl_str_mv Linear IgA bullous dermatosis: report of an exuberant clinical case and literature review
Dermatose bolhosa IgA Linear: relato de um caso exuberante e revisão da literatura
title Linear IgA bullous dermatosis: report of an exuberant clinical case and literature review
spellingShingle Linear IgA bullous dermatosis: report of an exuberant clinical case and literature review
Pereira, Sandra
Case Reports
title_short Linear IgA bullous dermatosis: report of an exuberant clinical case and literature review
title_full Linear IgA bullous dermatosis: report of an exuberant clinical case and literature review
title_fullStr Linear IgA bullous dermatosis: report of an exuberant clinical case and literature review
title_full_unstemmed Linear IgA bullous dermatosis: report of an exuberant clinical case and literature review
title_sort Linear IgA bullous dermatosis: report of an exuberant clinical case and literature review
author Pereira, Sandra
author_facet Pereira, Sandra
Martins, Alexandra
Oliveira, Teresa
Monteiro, Virgínia
author_role author
author2 Martins, Alexandra
Oliveira, Teresa
Monteiro, Virgínia
author2_role author
author
author
dc.contributor.author.fl_str_mv Pereira, Sandra
Martins, Alexandra
Oliveira, Teresa
Monteiro, Virgínia
dc.subject.por.fl_str_mv Case Reports
topic Case Reports
description Introduction: Linear IgA dermatosis (LIGAD) is a rare acquired disease, with a probable autoimmune origin. Its differential diagnosis involves other bullous dermatosis. Case Report: A previously healthy 12-month-old male was observed at the Emergency Department due to a 4-day itchy vesiculobullous rash in the perineal region, lower abdomen, hands, and feet. Analytical study was normal. Flucloxacillin and hydroxyzine were initiated without improvement. New (some of which confluent) lesions, erosions, and serohematic crusts developed on the periphery of previous lesions. A skin biopsy was performed at this time, revealing a subepidermal blister with neutrophilic infiltrate at histological examination. Direct immunofluorescence uncovered linear IgA deposits along the basement membrane. Lesion remission occurred without further therapeutic measures. Discussion: Although clinically exuberant, LIGAD is usually a self-limited disease. A high degree of suspicion is important, since immunofluorescence is diagnostic and pathognomonic, avoiding late diagnosis, unnecessary treatments, and parental anxiety.
publishDate 2019
dc.date.none.fl_str_mv 2019-01-02T00:00:00Z
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
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dc.identifier.uri.fl_str_mv https://doi.org/10.25753/BirthGrowthMJ.v27.i4.13021
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dc.language.iso.fl_str_mv eng
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dc.publisher.none.fl_str_mv Centro Hospitalar Universitário do Porto
publisher.none.fl_str_mv Centro Hospitalar Universitário do Porto
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repository.name.fl_str_mv Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
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