PMC5421084_01
(a) Vasculitic rash with a distinct circinate pattern. (b) Photomicrograph of skin biopsy demonstrating inflammation affecting the superficial and deep dermal small vessels, comprising predominantly mononuclear inflammatory cells, but with focal destruction of the vessel walls with fibrin deposition, red cell fragmentation, and scattered nuclear fragments (haematoxylin and eosin, original magnification x100).
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A three-year-old Caucasian boy was investigated for diffuse vasculitic skin lesions (Figure 1(a)). Physical examination revealed macrocephaly (head circumference > 97.5th centile) and an unusual vasculitic rash with a distinct circinate pattern (Figure 1(a)). A skin biopsy revealed thrombotic, lymphocytic vasculitis with an absence of immune-complex deposition on immunofluorescence (Figure 1(b)).