CRP and Splenomegaly: She developed arthralgias without apparent arthritis and intermittent fever (up to 38.5°C) at 21 and 23 months, respectively, splenomegaly and permanently increased inflammatory parameters (CRP 50-110 mg/l) were noted and the patient was treated for suspected systemic JIA with pulse CS and methotrexate and later cyclosporine A. With CS tapering intermittent fever and joint pain recurred, livedo racemosa was noted and a defect in B-cell differentiation without hypogammaglobulinaemia was identified (28 months).