Extreme Peripheral Eosinophilia in a Patient With a Skin Rash and Abnormal Chest Imaging

Abstract

INTRODUCTION: A unique case of lung disease and extreme eosinophilia. CASE PRESENTATION: A 56-year-old Caucasian male with epilepsy and 3 months history of asthma is hospitalized for skin purpura and arthralgia. He had cough, dyspnea on exertion, fever with significant constitutional symptoms. His medications are lamotrigine, montelukast and inhaled bronchodilators. Physical examination positive for fever, bilateral nasal turbinate hypertrophy, bilateral pitting ankle edema, and palpable purpura of the legs. Laboratory studies show leukocytosis with extreme eosinophilia (70%, absolute eosinophil count (AEC) of 21.2 k/cmm). He developed acute oliguric renal failure. Urinalysis positive for proteinuria and hematuria. Rheumatogical work up showed negative C-ANCA, P-ANCA (antineutrophil cytoplasmic autoantibody), viral hepatitis panel, and normal C3/C4 complement levels. Chest computerized tomography (CT) showed bilateral pulmonary ground-glass opacities (GGO). Renal core needle biopsy revealed pauci-immune crescentic glomerulonephritis with fibrinoid necrosis and acute eosinophilic interstitial nephritis. Skin biopsy had vasculitis with eosinophilic inflammatory cell infiltrate. The clinical picture was suggestive of Churg-Strauss Syndrome. He was given pulse steroid therapy with methylprednisolone and intravenous cyclophosphamide. The peripheral eosinophil count reduced to 3% (600k/cmm) within 31 hours. He was discharged on maintenance regimen. Repeat chest CT showed complete resolution of the bilateral GGO three month later. The patient has experienced clinical remission for 1.5 years now. DISCUSSION: The presence of asthma, eosinophilia, pulmonary infiltrates, vasculitis strongly suggested the diagnosis of Churg-Strauss Syndrome. In literature review, mean AEC in Churg-Strauss Syndrome was 7.1, highest reported as 44k/cmm). Other etiologies for GGO with eosinophilia include: A) Tropical filarial pulmonary eosinophilia (mean 15.6, highest reported 80 k/cmm), B) Idiopathic hypereosinophilic syndrome (AEC mean 6.6 K/cmm, highest reported 400 k/cmm), C) idiopathic chronic eosinophilic pneumonia (AEC mean 5.5K/cmm, highest reported 37.8 k/cmm, D) allergic bronchopulmonary aspergillosis(mean AEC 0.8 , highest reported 14.1 k/cmm) Careful clinical and pathological correlation is necessary to establish the correct diagnosis CONCLUSIONS: This patient’s eosinophilia (70%) is extreme but not unforeseen for Churg- Strauss syndrome.