A case of Churg-Strauss syndrome associated with antiphospholipid antibodies

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.

Figure 1

a, b. Cutaneous lesions in a patient with Churg-Strauss syndrome. Erythematous crusted papules on dorsal hands and fingers on presentation evolving to subsequent development of cyanosis, gangrene involving the right hand fingers one month later (a). Note resolution of dorsal hand skin lesions and development of progressive distal fingertip dry gangrene six months after initial presentation (b).

Figure 2

a,b. Histology of dorsal hand skin lesions on admission shows angiocentric and interstitial dermal infiltrates composed of neutrophils, neutrophil fragments, eosinophils and fibrin deposition in vessel walls (hematoxylin-eosin), magnification 4x (a) and 10x (b). Figure 2 c,d. Histology of sigmoid colon mesentery showing extensive vasculitis of medium sized blood vessels, prominent vasculocentric inflammation, fibrinoid necrosis, eosinophils (intravascular and extravascular) and neutrophils, magnification 20x (c) and 40x (d).