By Gary M. White, MD
Churg-Strauss syndrome (CSS), aka eosinophilic granulomatosis with polyangiitis, is a multi-organ systemic vasculitis characterized by tissue and blood eosinophilia.
- Asthma, a peak, peripheral eosinophil count in excess of 10%, and a dermal infiltrate of eosinophils help distinguish this ANCA-positive systemic vasculitis from granulomatosis with polyangiitis and microscopic polyangiitis.
- Glomerulonephritis and a mono or polyneuropathy may occur.
- CSS is a necrotizing vasculitis that affects the small and medium sized vessels.
Skin changes are variable and include an erythematous maculopapular eruption, hemorrhagic lesions, sometimes accompanied by necrosis, and cutaneous and subcutaneous nodules.
In one study [JAAD 1997;37;199], 40% of patients had skin findings and the most frequent were purpura and petechiae on the legs and cutaneous nodules and papules on the elbows.
Consultation with a rheumatologist is in order. In the past, prednisone e.g., 60-100 mg/day, has been used first. Cyclophosphamide has been added if needed. Mycophenolate mofetil was used successfully.
A 53-year-old patient presented with a history of recently diagnosed and worsening asthma with nasal polyposis. A mononeuritis and a vasculitic rash were noted and blood screen showed hypereosinophilia and immunoglobulin E (IgE) elevation.
BMJ Case Rep. 2011;
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