Chronic Periaortitis (Retroperitoneal Fibrosis) Concurrent with Recurrent Cutaneous Eosinophilic Vasculitis

Figure 2

Chronic periaortitis presenting as recurrent cutaneous eosinophilic vasculitis (RCEV). (a) Skin biopsy showing under a focally slight spongiotic epidermis edema and moderately dense perivascular and interstitial inflammatory cell infiltrates confined to the upper dermis (hematoxylin & eosin; ×40). (b) Histopathology of skin lesion (detail) showing the inflammatory infiltrate consisting of lymphocytes, monocytes, and plentiful eosinophils. Altered vessels with endothelial swelling, intraluminal fibrin, a few eosinophils within vessel walls, and eosinophilic dust around them were featured. Leukocytoclasis or overt vascular necrosis was not seen (hematoxylin & eosin; ×160). (c) Contrast-enhanced CT scan (portal phase) revealed a mantle of a soft-tissue mass enveloping the aorta, consistent with chronic periaortitis (retroperitoneal fibrosis). Typically, the aorta is encased but not displaced by this process. Arrow: right hydroureter.