PMC3504283_01
Chronic periaortitis presenting as recurrent cutaneous eosinophilic vasculitis (RCEV). (a, b) Urticarial, partly annular skin lesions of dorsal and medial aspects of right wrist-hand region. (c) Urticarial-erythematous skin lesions of the right leg; note signs of blood extravasations in healed lesions.
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On admission, physical examination was unremarkable except for multiple polycyclic-annular, erythematous papules, and plaques mainly affecting her extremities, some with signs of central clearing and scales (Figures 1(a)-1(c)). Many lesions initially simulated wheals, yet they grew slowly over 1-2 weeks to fade subsequently leaving behind grayish-colored maculas (Figure 1(c)).
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; x40). (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; x160). (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.
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A lesional skin biopsy showed a moderately dense perivascular and interstitial inflammatory cell infiltrate in the upper dermis (Figure 2(a)), consisting of lymphocytes, monocytes, and plentiful eosinophils. Endothelial swelling, intraluminal fibrin, eosinophils within vessel walls and eosinophilic dust around them were featured, consistent with the diagnosis "eosinophilic vasculitis" (Figure 2(b)).