MICROSCOPIC POLYANGIITIS WITH EOSINOPHILI A - AN OVERLAP SYNDROME OR A SEPARATE DISEASE ENTITY - CASE-REPORT AND LITERATURE-REVIEW

Systemic vasculitides are potentially life-threatening diseases. Early and appropriate diagnosis based on case history, clinicopathological features, and laboratory parameters, such as the presence of anti-neut rophil cytoplasmic antibodies (ANCA), is crucial for starting appropri ate and, often,life-saving therapeutic measures. We report a 50-year-o ld female patient who presented with fever, arthralgias and hemoptysis . Skin signs included disseminated hemorrhagic pustules, ulcerations o f oral and genital mucosa, subcutaneous nodules on arms and legs, and a pyoderma gangrenosum-like lesion on the right leg. laboratory invest igations revealed a peripheral eosinophilia and a positive cANCA titer . Histopathologic analysis of various biopsy specimens showed a granul omatous vasculitis in the subcutis, a nongranulomatous vasculitis with massive eosinophil infiltration in the lungs, and a segmental, necrot izing glomerulonephritis in the kidneys. Differential diagnosis includ ed Wegener's granulomatosis, microscopic polyangiitis (MPA) and Churg- Strauss syndrome. MPA was diagnosed based on clinical and histopatholo gical criteria. An interesting feature of this case was marked periphe ral and tissue eosinophilia. Therapy consisted of cyclophosphamide and methylprednisolone. The patient went into a long-lasting clinical rem ission one month after starting therapy.