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

  • Sandra Pereira Pediatrics Department, Hospital Pediátrico Integrado. Centro Hospitalar Universitário São João
  • Alexandra Martins Pediatrics Department, Centro Hospitalar Entre Douro e Vouga
  • Teresa Oliveira Pediatrics Department, Centro Hospitalar Entre Douro e Vouga
  • Virgínia Monteiro Pediatrics Department, Centro Hospitalar Entre Douro e Vouga
Keywords: Linear IgA bullous dermatosis, pediatrics, skin diseases, vesiculobullous

Abstract

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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Published
2019-01-02
Section
Case Reports