Leukocytoclastic vasculitis in children: clinical characteristics, subtypes, causes and direct immunofluorescence findings of 56 biopsy-confirmed cases

J Eur Acad Dermatol Venereol. 2017 Mar;31(3):544-549. doi: 10.1111/jdv.13952. Epub 2016 Sep 19.

Abstract

Background: Leukocytoclastic vasculitis (LCV) in children is a complex group of conditions.

Objectives: This study presents the demographics, clinical features, direct immunofluorescence (DIF) results and suspected aetiologies of 56 biopsy-confirmed cases of leukocytoclastic vasculitis in children.

Methods: Retrospective review of 56 children seen at Mayo Clinic in Rochester, Minnesota, from 1993 to 2013 with clinical features and cutaneous biopsy consistent with LCV.

Results: Twenty-seven (48%) cases were found to be due to IgA vasculitis (Henoch-Schonlein purpura). The remaining cases were found to be due to cutaneous small-vessel vasculitis (n = 19, 34%), urticarial vasculitis (n = 5, 9%), ANCA-associated vasculitis (n = 4, 7%) and acute haemorrhagic oedema of infancy (n = 1, 2%). IgA vasculitis was found to be associated with abdominal pain (P = 0.008), whereas the non-IgA vasculitis group was associated with headache (P = 0.052). Children with IgA vasculitis had palpable purpura (P = <0.001), petechia (P = 0.057), vesicles (P = 0.009) and involvement of the buttock (P = 0.004) more frequently than the non-IgA vasculitis group. On DIF, perivascular IgA was positive in IgA vasculitis compared to non-IgA vasculitis cases (P = <0.001), the other conjugates were similar between the two groups.

Conclusion: The most common subtype of biopsy-confirmed LCV in children is IgA vasculitis. Clinical features, exam characteristics and DIF results can be helpful in determining the subtype of cutaneous vasculitis in children.

MeSH terms

  • Abdominal Pain / etiology
  • Adolescent
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / complications*
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / diagnosis*
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / etiology
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / metabolism
  • Blister / etiology
  • Child
  • Child, Preschool
  • Fatigue / etiology
  • Female
  • Fluorescent Antibody Technique, Direct
  • Headache / etiology
  • Humans
  • IgA Vasculitis / complications*
  • IgA Vasculitis / diagnosis*
  • IgA Vasculitis / etiology
  • IgA Vasculitis / metabolism
  • Immunoglobulin A / metabolism
  • Infant
  • Male
  • Purpura / etiology
  • Retrospective Studies
  • Vasculitis, Leukocytoclastic, Cutaneous / complications*
  • Vasculitis, Leukocytoclastic, Cutaneous / diagnosis*
  • Vasculitis, Leukocytoclastic, Cutaneous / etiology
  • Vasculitis, Leukocytoclastic, Cutaneous / metabolism

Substances

  • Immunoglobulin A