A case of Churg-Strauss syndrome associated with antiphospholipid antibodies

J Am Acad Dermatol. 2007 Apr;56(4):701-4. doi: 10.1016/j.jaad.2006.09.028. Epub 2006 Dec 18.

Abstract

Churg-Strauss syndrome (CSS) is a systemic vasculitis affecting both small- and medium-sized blood vessels, almost invariably affecting the lung, and frequently associated with cutaneous involvement. Microvascular vaso-occlusion leading to digital gangrene is not a feature of CSS. We report an unusual case of a patient with CSS with antiphospholipid antibodies who developed severe digital gangrene in addition to cutaneous vasculitis. The presence of antiphospholipid antibodies is not a feature usually seen in association with CSS. While the full clinical spectrum of CSS is still being defined, the identification of additional features associated with this syndrome might help to better understand the pathogenesis of the disease and to have an impact on both management and prognosis.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural

MeSH terms

  • Antibodies, Antiphospholipid / blood
  • Antibodies, Antiphospholipid / immunology*
  • Azathioprine / therapeutic use
  • Biopsy, Needle
  • Churg-Strauss Syndrome / drug therapy
  • Churg-Strauss Syndrome / immunology*
  • Churg-Strauss Syndrome / pathology*
  • Disease Progression
  • Drug Therapy, Combination
  • Fingers
  • Gangrene / diagnosis
  • Gangrene / drug therapy
  • Humans
  • Immunohistochemistry
  • Male
  • Middle Aged
  • Prednisone / therapeutic use
  • Prognosis
  • Risk Assessment
  • Severity of Illness Index
  • Skin / pathology*
  • Treatment Outcome

Substances

  • Antibodies, Antiphospholipid
  • Azathioprine
  • Prednisone