Churg-Strauss syndrome

Background The Churg-Strauss syndrome, also known as allergic granulomatosi s with angiitis, is a rare necrotizing vasculitis with unknown pathogenesis . The necrotizing granulomatous vasculitis involves small and medium-sized arteries, capillaries and veins, leading to the characteristic changes of i ntra- and extravascular, eosiniphilic granulomas, accompanied by clinically symptoms: bronchial asthma, hypereosinophilia and fever. Ocular manifestat ions are rarely reported. Case report A 53-year-old woman suffered from bronchial asthma, relapsing l ung infiltrates and sinusites since 1994. In August 1997 she suddenly discl osed vasculitic skin manifestations on both legs and a mononeuritis of the left peroneus nerve. At the same time visual acuity of the right eye decrea sed, before she had shown some attacks of amaurosis fugax. The funduscopy s howed a central retinal artery occlusion. Laboratory findings: blood eosino philia of 20%, elevated IgE value to 396 kU/l (normal value <120 kU/l), and negative parameters for antineutrophil cytoplasmatic antibodies (p- and c- ANCA). Conclusion The clinical and laboratory findings are characteristic signs fo r the Churg-Strauss syndrome. Without such typical manifestations the histo logic examination leads to the diagnosis and helps to differentiate this di sease from other necrotizing vasculitides, e.g. panarteriitis nodosa or Weg ener's granulomatosis.