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Vasculitis is defined by a state of inflammation of the blood vessel walls. It can result in formation of either aneurysms or stenosis with ischemia and necrosis. The heterogeneity of clinical presentation depends on the type and size of the vessels involved, which leads to different patterns of organ damage. The skin is usually involved but not necessarily as a first manifestation of disease. Indeed, significant organ damage may occur at any time in the disease course and may precede other symptoms with significant morbidity and mortality, especially in case of diagnostic delay. The most common vasculitides in childhood are IgA-associated vasculitis (Schönlein-Henoch purpura) and Kawasaki disease. Other vasculitides are much rarer, often arising with insidious symp- toms that make them life-threatening. This paper aims to review the literature on paediatric vasculitides providing a practical guide to disentangle between different diagnoses. It also provides an overview that underlines the key differences in vasculitis presentation, management and follow-up.

A 7-year-old girl with a longstanding history of recurrent pneumonia, arthritis and unsteady gait was finally diagnosed with ataxia-telangiectasia. The classical bulbar conjunctival telangiectasia was present and helped to make the diagnosis.