Lestre, Sara; Serrão, Vasco; Joao, Alexandre; Pinheiro, Sofia; Lobo, Lurdes
April 2009
Acta Reumatológica Portuguesa;2009, Vol. 34 Issue 2, p281
Academic Journal
Churg-Strauss syndrome (CSS), also known as allergic granulomatous angiltis, is a rare disorder characterized by the presence of asthma, eosinophilia and small-to-medium sized vessels vasculitis. Vasculitis commonly affects lung, heart, skin and peripheral nerves. The authors report a case of a 47-year-old woman that was admitted with a 1-week history of painful purpuric lesions affecting extensor surfaces of lower extremities. She had a 7 years-long medical history of asthma, paranasal sinusitis and allergic rhinitis. Laboratorial tests revealed leucocytosis, peripheral blood eosinophilia and elevated inflammatory parameters. Antineutrophil cytoplasmic antibodies were negative. Skin biopsy was consistent with necrotizing vasculitis with perivascular eosinophil-rich infiltrate. Electromyogram and nerve conduction studies showed asymmetrical peripheral polyneuropathy. Churg-Strauss syndrome diagnosis was supported by clinical and histopathological findings. High-dose oral steroid therapy led to a dramatic clinical improvement. Skin manifestations can be the presenting feature of Churg-Strauss syndrome. Early recognition is essential for appropriate treatment and prevention of irreversible organ damage.


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