Rare vasculitic syndromes

Vasculitis can and does occur in childhood. Apart from the common vasculitides (Henoch-Schönlein purpura, hypersensitivity angiitis and Kawasaki disease) there are a number of important but comparatively rare disorders affecting children. These include macroscopic and microscopic polyarteritis, cutaneous polyarteritis, Wegener's granulomatosis, Churg-Strauss syndrome, primary angiitis of the central nervous system, hypocomplimentaemic urticarial vasculitis, vasculitis associated with various connective tissue disorders, Takayasu's disease and vasculitis associated with conditions such as Behcet's syndrome, familial Mediterranean fever and Cogan's syndrome. Distinguishing these conditions from other disorders is often difficult and requires clinical acumen and appropriate investigative procedures. With modern therapeutic agents it is possible to implement appropriate therapy but in spite of this, there remains a not inconsequential morbidity and mortality.