A Jigsaw Puzzle of Celiac Sprue: Solved

Abstract

Celiac disease represents a prototypical malabsorptive disorder of the small bowel. Although its etiopathogenesis is still incompletely understood, the striking clinical and histologic features have been well described in the literature. Perhaps less well known are the classic radiological findings associated with this disorder. We describe one such finding in a patient who was subsequently diagnosed with celiac disease. A 46-year-old man with comorbidities of hypothyroidism and compensated cirrhosis secondary to chronic hepatitis-C presented with abdominal pain, bloating and diarrhea. Physical examination was unremarkable barring striking facial maculopapular rash (Figure 1) which the patient reported intense itchiness. A diagnostic esophagogastroduodenoscopy (EGD) was grossly normal but duodenal biopsies demonstrated increased intra-epithelial lymphocytes (Figure 2). A subsequent small bowel follow through demonstrated flocculation of oral contrast and ‘jejunalization’ of the ileum with increased bowel wall folds suggestive of malabsorptive small bowel pathology such as tropical or celiac sprue (Figure 3). Given the comprehensive picture drawn from the presence of comorbid condition like hypothyroidism, physical examination finding of facial rash consistent with Dermatitis Herpetiformis, suggestive small bowel histology and the singular fluoroscopic findings- a diagnosis of celiac sprue was made. The patient was put on gluten free diet with significant improvement in his symptoms. Dermatitis herpetiformis is a maculopapular &/or vesicular cutaneous rash that is sometimes seen in patients with gluten sensitivity. It typically involves the extensor surfaces (knees, elbows), buttocks and posterior scalp; and less commonly the face as seen in our patient. The natural history of celiac disease includes loss of villi and hypertrophy of crypts, resulting in fluid malabsorption and hypersecretion respectively. The resulting chronic excess of fluid gives rise to the small bowel malabsorption pattern (MABP) described in barium studies of patients with celiac disease, whose salient features include flocculation. Flocculation represents a radiological artifact caused by the precipitation of tiny hyperattenuating clusters of barium within hypersecreted fluid. Barium flocculation is seen in up to 26% of celiac sprue patients but is not specific. MABP also is characterized by reversal of the jejunal-ileal fold pattern as seen in our patient.Figure 1Figure 2Figure 3