Inflammatory pseudotumor of the anterior orbit. A symptom in allergic granulomatous angiitis (Churg-Strauss syndrome).

Allergic granulomatosis accompanied by angiitis (Churg-Strauss syndrome) constitutes an unusual disorder which is characterized clinically by bronchial asthma and hypereosinophilia, accompanied by systemic symptoms of histopathologically and by necrotizing vasculitis, extravascular granulomas and tissue infiltration by eosinophils. We report a case of a 4 year-old child presenting with acute pneumonia and deteriorated general condition. The biopsy of an inflammatory pseudotumor of the right anterior orbit revealed necrotizing vasculitis, extravascular epitheloid granuloma and eosinophilic tissue infiltration. These are characteristic changes of allergic granulomatous vasculitis (Churg-Strauss syndrome. Laboratory findings were characterized by blood eosinophilia, increased IgE value and the presence of antineutrophil cytoplasmic ant antibodies (p-ANCA). Local and systemic manifestations quickly regressed after parenteral application of corticosteroids. Two exacerbations of inflammatory pseudotumor occurred after reduction of the corticosteroid dosage. Systemic vasculitic syndromes are rare in childhood. Orbital manifestations in Churg-Strauss syndrome in childhood have never been reported.