Severe hemorrhagic bullous lesions in Henoch Schonlein purpura: three pediatric cases and review of the literature

Rheumatol Int. 2010 Aug;30(10):1355-9. doi: 10.1007/s00296-009-1055-8. Epub 2009 Jul 16.

Abstract

Henoch Schonlein purpura (HSP) is a small-sized blood vessels vasculitis, resulting from immunoglobulin A-mediated inflammation. It is the most common acute systemic vasculitis in childhood and mainly affects skin, gastrointestinal tract, joints and kidney. Skin lesions, usually presenting as erythematous maculopapules, petechiae, and purpura, often involve lower extremities and buttocks, but may also extend to the upper extremities, face and trunk. Conversely to adults, hemorrhagic bullous evolution has been seldom described in childhood. The pressure is likely a factor into the pathogenesis of bullae. We report on three new pediatric cases of HSP with hemorrhagic bullous skin lesions, and a review of the literature. Bullous evolution represents an unusual, but well-recognized cutaneous manifestation that may be a source of diagnostic dilemma, but does not seem to have any prognostic value in the outcome of HSP.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Azathioprine / therapeutic use
  • Blister / complications
  • Blister / drug therapy
  • Blister / pathology*
  • Child
  • Female
  • Glucocorticoids / therapeutic use
  • Hemorrhage / complications
  • Hemorrhage / drug therapy
  • Hemorrhage / pathology*
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Male
  • Prednisone / therapeutic use
  • Prognosis
  • Purpura, Schoenlein-Henoch / complications
  • Purpura, Schoenlein-Henoch / drug therapy
  • Purpura, Schoenlein-Henoch / pathology*
  • Remission, Spontaneous
  • Skin / pathology*
  • Treatment Outcome

Substances

  • Glucocorticoids
  • Immunosuppressive Agents
  • Azathioprine
  • Prednisone