Ceroid deposition in the muscularis of the small intestine, associated with regional enteritis

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CEROID pigment deposited in the smooth muscle of the small intestine in humans is an unusual finding. Characteristically the pigment is only deposited after prolonged vitamin E deficiency.1 Of 141 published cases,1–12 in 127 cases there was microscopic evidence and in 14 cases gross evidence,1–8 but only rarely has clinical correlation been possible.

A variety of diseases, such as cystic fibrosis, chronic pancreatitis,4, 12 nontropical sprue,3, 7 Whipple’s disease,6, 8 celiac disease,10 biliary atresia,10 Laennec’s cirrhosis10, 12 portal cirrhosis,10 biliary cirrhosis associated with chronic pancreatitis,12 profound nutritional imbalance,1–3, 12 idiopathic hypo-proteinemia,5 idiopathic steatorrhea,7 jejunal diverticulosis,7 and terminal ileitis associated with jejunal diverticulosis9 have been reported in which ceroid pigment has been found in the muscularis propria of the small intestine.

Although Paulley9 reported a case of terminal ileitis and jejunal diverticulosis in which golden-brown pigment granules and the smooth-muscle cells of the small bowel were found on microscopic examination, he did not associate the granules with ceroid. We wish to report what we believe to be the second case of ceroidosis associated with regional enteritis, and the fifteenth case in which the ceroid pigment was evident on gross examination of the small bowel.

Report of a case

A 51-year-old woman was first examined at the Cleveland Clinic in 1957 because of 8 t o 10 loose (not bloody) bowel movements per day for more than 15 years. Her medical history revealed that between 1942 and 1944 she had undergone three abdominal operations for small-bowel obstruction and abscess formation.



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