A case of ANCA positive Churg-Strauss syndrome with widespread bloody bullae and leg skin ulcer

Abstract

    A 57-year-old woman was admitted to our hospital because of CSS (Churg-Strauss syndrome) with widespread bloody bullae and leg skin ulcer. She had been treated for asthma since she was 45. From the middle of August, she had repeated low grade fever (37°C) podarthralgia and urticaria-like eruption on both lower limbs. From the end of August, she presented with high fever (39°C), purpura and bloody bullae on her hands and legs. Then, she was admitted to our hospital on September 9th. Laboratory findings revealed eosinophilia -(1100/mm³), high levels of serum CRP (10.7 mg/dl) and nonspecific IgE elevation (943 IU/ml). MPO-ANCA titer increased to 920 EU (normal range＜10 EU), and ulinalysis showed proteinuria and microhematuria. Skin biopsy of the leg purpura demonstrated leukocytoclastic vasculitis with increased eosionophil infiltration in dermis middle class. She was diagnosis as having Churg-Strauss syndrome and was treated with prednisolone including steroid-pulse therapy, Although the therapy improved fever and CRP level. Purpura and bloody bullae changed to widespread skin ulcers. We report as a rare case of MPO-ANCA positive Churg-Strauss syndrome with widespread bloody bullae and leg skin ulcer.