Sindrome di Churg-Strauss

We report the case of a 54-year-old man admitted to the emergency unit of our hospital with severe asthma, arthralgias, fever, paresthesias and asymptomatic purpuric lesions on the upper and lower extremities. His past history was remarkable for recurrent episodes of asthma, nasal polyposis, hypertensive cardiopathy and comitial attacks. Laboratory findings were leukocytosis with prominent eosinophilia, elevated erythrocyte sedimentation rate and increase of anti-neutrophil cytoplasmic antibodies (p-ANCA). Urine analysis revealed mild proteinuria. A chest radiograph revealed bilateral pulmonary infiltrates; multiple necrotic areas in the kidneys and liver were detected by tomographic scan and nuclear magnetic resonance. Histopathologic examination of a purpuric papule was striking for the presence of foci of basophilic collagen degeneration surrounded by histiocytes with many neutrophils and eosinophils. Based on the clinical, histopathologic and laboratory findings, the diagnosis of Churg-Strauss syndrome was made and prednisone 1mg/kg/die was started with dramatic response. Churg-Strauss syndrome is an uncommon multi-system disease with clinical and histopathologic features that sometimes overlap with other ANCA-vasculitides. Diagnosis is often difficult because the clinicopathologic findings evolve according to the different phases of the disease. The skin holds particular significance because it serves as a useful site for histopathologic diagnosis.