Mimics of Vasculitis: Vascular Ehlers-Danlos Syndrome Masquerading as Polyarteritis Nodosa

Abstract

To the Editor: A 29-year-old African American man presented to the emergency department following a witnessed generalized seizure, his first. He provided a history of 3 days of abdominal discomfort prior to presentation, associated with constipation. History was remarkable for alprazolam abuse that he recently discontinued, and inguinal hernia repair as a child. There was no significant family history. Physical examination, laboratory studies, and computed tomography (CT) scan of the head were normal. A lumbar puncture was not performed. A second witnessed seizure prompted admission. Over the next 2 days he had no further neurological events but complained of persistent abdominal discomfort. On the third hospital day he developed a fever of 38.5°C associated with leukocytosis and decreasing hemoglobin, but normal chemistry results, amylase, and lipase. CT scan of the abdomen/pelvis revealed a ruptured splenic artery aneurysm with intraabdominal hemorrhage, splenic vein thrombosis, celiac artery aneurysm, bilateral external iliac artery aneurysms, renal artery aneurysm, and multiple splenic and renal infarcts. CT angiography further delineated the extent of the vascular abnormalities (Figure 1). Figure 1. Sequence of reconstructed CT … Address correspondence to Dr. R.A. Badawi, Department of Cardiology, Ochsner Clinic Foundation, 1514 Jefferson Highway, New Orleans, LA 70121. E-mail: rbadawi{at}msn.com