Acute Intermittent Porphyria - an Unexpected Association in a Patient with Newly Diagnosed Crohn’s Disease

Abstract

The association of Crohn‘s disease (CD) with acute intermittent porphyria (AIP), both without a family or personal pathological history, is a very rare clinical possibility. We present the case of a 23-year-old male diagnosed on the same admission with ileal CD and with an AIP crisis. The diagnosis was challenging as the symptoms overlapped. Crohn’s disease was complicated with intestinal occlusion and sepsis; the inflammatory, metabolic and septic changes represented the trigger factor for the first AIP seizure. The neurological symptoms were the key element for AIP diagnosis. The presence of atypical extra-intestinal manifestations in CD patients should raise also the possibility of AIP.

Polybacterial Iliopsoas Muscle Abscess as an Indication for Early Diagnosis of Crohn\'s Disease

“…The clinical manifestations of a CD case localized to the appendix are similar to those of acute appendicitis, and a diagnosis of CD is possible only by histopathological examinations [ 20 ]. The reported atypical or rare extra-gastrointestinal signs of CD are as follows: complication of superior mesenteric vein obstruction and protein-losing enteropathy [ 21 ]; thrombosis in the portal vein and splenic vein, with diffuse abdominal lymphadenopathy requiring differentiation from malignant lymphoma [ 22 ]; acute intermittent porphyria [ 23 ]; and presacral abscess [ 24 , 25 ]. The present case was an atypical case of CD because of the lack of systemic and gastrointestinal signs other than fever.…”