Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome or allergic granulomatosis and angiitis, is a rare small- and medium-vessel vasculitis. This disease was first described by American pathologists Jacob Churg and Lotte Strauss in 1951.1 Although the vasculitis is often not apparent in the initial phases of the disease, EGPA can affect any…
Clinical Vignette A 45-year-old woman with long-standing asthma and chronic sinusitis has new-onset peripheral neuropathy, arthralgias, fatigue, progressive dyspnea and a nonproductive cough. She has never smoked and has no environmental exposures. Her medications include an albuterol metered-dose inhaler (which she uses daily); an inhaled corticosteroid, montelukast; and ibuprofen (which she takes occasionally). She is…
In this third article in the series, we talk with Philip Seo, MD, MHS, about eosinophilic granulomatosis with polyangiitis (EGPA).
Eosinophilic granulomatosis with polyangiitis (EGPA) is an anti-neutrophil cytoplasmic antibody-associated vasculitis typically characterized by asthma, peripheral eosinophilia and medium- to small-vessel necrotizing vasculitis. Cutaneous manifestations in EGPA are diverse. Palpable purpura is the most common presentation, but urticaria, erythematous macules and papules, livedo reticularis, digital necrosis and cutaneous nodules have also been described.1 Non-hemorrhagic bullae…