ANCA-positive vasculitis of the skin and kidneys associated with acne conglobata

A 19 year old man with severe acne conglobata and ulcerated pyoderma gangraenosum-like skin lesions on the face was first treated with isotretinoin (Roaccutan((R))), then immunosuppressively with prednisolone, diaminocliphenylsulfone (Dapson-Fatol((R))) and mycophenolate mofetil (Celicept((R))). Under a daily maintenance dose of immunosuppressive treatment with 2.5 mg prednisolone and I g mycophenolate mofetil, weakness, muscle and joint aches appeared. Due to proteinuria, haematuria and an elevated antineutrophil cytoplasmic antibody (cANCA) and the histological detection of a leukocytoclastic vasculitis, the diagnosis of cANCA positive vasculitis of the skin and kidneys was established. Therapy with cyclophosphamide (Endoxan((R))) along with prednisolone was effective. An exact classification of this disease process was not possible.