INTRODUCTION Idiopathic systemic vasculitis represents a group of clinical entities having non-specific etiology with the common characteristic of acute or chronic inflammatory compromise of the small and large vessels walls, associated with fibrinoid necrosis. OBJECTIVES To describe the most common inflammatory vascular diseases in a long historical cohort of patients from San Juan de Dios H...

Background: Primary Systemic Vasculitides (PSV) constitute a heterogeneous group of rare and potentially life-threatening autoimmune diseases, characterized by varying degree inflammatory response, leading to local or generalized vascular disease. Vessel involvement accounts for the micro- macrovascular complications disease, along with classic risk factors including, among others age chronic u...

Childhood primary angiitis of the central nervous system (cPACNS) is a rare and a potentially fatal cause of childhood stroke. The disease poses a diagnostic dilemma for the clinicians due to overlapping and varied clinical manifestations such as headache, focal acute neurological deficits, cognitive impairment, or encephalopathy. We report a young boy who presented with low-grade fever and hea...

Acute urticarial lesions may display central clearing with ecchymotic or haemorrhagic hue, often misdiagnosed as erythema multiforme, serum-sickness-like reactions, or urticarial vasculitis. We report a case of acute annular urticaria with unusual presentation occurring in a 20-month-old child to emphasize the distinctive morphologic manifestations in a single disease. Clinicians who care for c...

Background and Aims: The diagnosis of Churg-Strauss syndrome (CSS), an allergic granulomatosis involving smalland medium-sized vessels, requires the presence of at least four of six criteria including asthma, peripheral eosinophilia, and systemic vasculitis. The gastrointestinal manifestations have never been reviewed. Methods: The 75 articles in the English literature found by electronic searc...

Immunoglobulin A (IgA) vasculitis, formerly called Henoch-Schönlein purpura, is an immune complex vasculitis affecting small vessels with dominant IgA deposits. Clinical manifestations mainly involve cutaneous purpura, arthralgias and/or arthritis, acute enteritis and glomerulonephritis. IgA vasculitis is more common among children than adults, with more severe disease in adults. Gastrointestin...

Background Tsutsugamushi disease is an acute, febrile, infectious disease caused by Orientia tsutsugamushi. Several studies investigating the histopathologic findings of eschars in tsutsugamushi disease reported leukocytoclastic vasculitis and neutrophil infiltration as the major findings. However, these findings may result from secondary changes following tissue necrosis. The histopathologic f...

Extrameningeal complications of meningococcal septicaemia occur in about 11–19% of cases, and include myocarditis, acute renal failure, arthritis, pneumonia, skin gangrene, conjunctivitis, endocarditis, pericarditis, endophthalmitis, urethritis, WaterhouseFriderichsen syndrome, vasculitis, and digital ischaemia. We describe the use of a prostacyclin analogue in the treatment of cutaneous digita...

Henoch-Shönlein purpura (HSP) is an acute, self-limited, systemic, small vessel vasculitis, that induces skin lesions, arthritis and abdominal pain. Palpable purpura is the most common manifestation in pediatric patients with HSP. We present an atypical case of HSP in a young patient and report successful treatment of the atypical skin lesions, while avoiding surgery.

Acute retinal necrosis (ARN) is a severe ocular syndrome consisting of a moderate-to-severe anterior uveitis, vasculitis, and vaso-occlusive retinal necrosis. It can occur in healthy individuals at any age, but reports of this condition in children are rare.