Linear IgA Bullous Dermatosis: A Series of 17 Cases

Actas Dermosifiliogr. 2019 Oct;110(8):673-680. doi: 10.1016/j.ad.2018.06.017. Epub 2019 Apr 20.
[Article in En, Spanish]

Abstract

Linear IgA bullous dermatosis is an acquired subepidermal immunoglobulin-mediated vesiculobullous disease. In this retrospective, observational, descriptive study, we describe the clinical characteristics, treatments, and outcomes of 17 patients with linear IgA bullous dermatosis. Two children had been vaccinated 2 weeks before the onset of symptoms, 2 had had bronco-obstructive respiratory symptoms, and 1 had received intravenous antibiotic therapy. We also observed an association with autoimmune hepatitis in one patient and alopecia areata in another. One boy had VACTERL association. Diagnosis was confirmed by histopathology and direct immunofluorescence. Sixteen patients were treated with dapsone, which was combined with oral corticosteroids in 8 cases and topical corticosteroids in two. Of note in this series was the occurrence of relapses in the perioral area coinciding with infections and vaccination, and the association between linear IgA bullous dermatosis and autoimmune hepatitis and VACTERL association.

Keywords: Chronic bullous dermatosis of childhood; Dermatosis ampollar IgA lineal; Dermatosis ampollar crónica de la infancia; IgA lineal; Linear IgA; Linear IgA bullous dermatosis.

Publication types

  • Observational Study

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Child
  • Child, Preschool
  • Dapsone / therapeutic use
  • Dermatologic Agents / therapeutic use
  • Female
  • Fluorescent Antibody Technique, Direct
  • Humans
  • Infant
  • Linear IgA Bullous Dermatosis* / diagnosis
  • Linear IgA Bullous Dermatosis* / drug therapy
  • Linear IgA Bullous Dermatosis* / etiology
  • Linear IgA Bullous Dermatosis* / pathology
  • Male
  • Retrospective Studies

Substances

  • Adrenal Cortex Hormones
  • Dermatologic Agents
  • Dapsone