A self-limited, leukocytoclastic vasculitis that presents as large urticarial plaques, fever, and edema in children between the ages of 4 months to 3 years.

Although the clinical presentation can be alarming, the condition has a benign clinical course.

Presumed to be an immune complex-mediated vasculitis.

Onset is preceded by a recent respiratory infection, a medication, or an immunization in three out of four cases.

Acute onset of large, urticarial or annular, targetoid, purpuric plaques found primarily on face, ears, and extremities (Fig. 2.27).

The trunk is relatively spared.

Tender edema on face (especially eyelids and ears) and/or extremities is common (Fig. 2.28).

Occasional petechiae, purpura, or necrotic lesions.

Fever is usually low grade and involvement of kidneys, joints, and gastrointestinal tract is rare.

Diagnosis is based on clinical recognition and the natural history of the condition.

A skin biopsy will reveal a leukocytoclastic vasculitis. Direct immunofluorescence is negative for IgA deposition in the majority of cases.