Diffuse muscular haemorrhage as presenting sign of juvenile systemic lupuserythematosus and lupus anticoagulant hypoprothrombinaemia syndrome

Lupus anticoagulants are closely related to systemic lupus erythemathosus ( SLE) and to thrombotic events. We describe a 12 year-old girl with a bilate ral intramuscular haemorrhage of the gastrocnemius muscles as her main init ial presentation of juvenile SLE. Laboratory work-up revealed lupus anticoa gulant-hypoprothrombinaemia syndrome (LAHS) with very low levels of factor II due to autoantibodies. She showed a good initial clinical and laboratory response to prednisone therapy, however steroid dependency developed. To t he best of our knowledge, this is the first case reported of juvenile SLE p resenting with LAHS.