Fever for > 5 days plus 4 of the following:
Bilateral bulbar conjunctival injection
Mucous membrane changes (injected pharynx, cracked lips, strawberry tongue)
Extremity changes (edema, erythema, or desquamation of hands or feet)
Cervical lymphadenopathy (at least one >1.5 cm)
Arthritis and arthralgia
Present in 7-25% of patients
Involves large or small joints
Causes sterile pyuria
Aseptic meningitis, facial nerve palsy, hearing loss
Abdominal pain, diarrhea, vomiting, hepatitis
Coronary artery aneurysms, myocardial dysfunction
Most common systemic vasculitis in children.
Deposition of IgA immune complexes.
Often a self-limited disease.
Occurs more often in fall, winter, and spring.
Rare in the summer.
About 50% of cases are preceded by URI’s.
Streptococcus is often implicated.
Vaccines, insect bites, viruses have also been reported as triggers.
Palpable purpura (100%)
In absence of thrombocytopenia or coagulopathy
Arthritis or arthralgia (75%)
Abdominal pain (50%)
Renal disease (21-50%)
Very rare systemic vasculitis of unknown etiology.
Affects blood vessels of all sizes.
Both arteries and veins
Clinical manifestations are similar in children and adults.
Characterized by recurrent, painful ulcers of the mouth and skin and uveitis.
GI symptoms (due to ulcers)
Normal ANA and RF
ESR/CRP may be elevated
Juvenile Systemic Sclerosis
Characterized by symmetrical fibrous thickening of skin and various internal organs.
Esophagus and GI tract, heart, lungs, kidneys
Skin changes – edema then tightening, thinning, atrophy
Raynaud’s – 70% at presentation
Pulmonary fibrosis and pulmonary hypertension are major causes of morbidity in children.