Pseudoobstruction in ceroidosis.
Diffuse deposition of ceroid pigment in the muscularis propria of the gastrointestinal tract in a patient with a long history of malabsorption of unknown origin is reported. The depostion of this waste pigment is not reversible and is related to prolonged depletion of vitamin E. Progressive dilatation and hypomotility of the entire gastrointestinal tract are demonstrated by radiographic studies and possibly related to infiltrate of ceroid pigment in the smooth muscle cell with resulting functional impairment. In the differential diagnosis of ceroidosis with other disease, scleroderma has the closest roentgenographic similarity. Pseudoobstruction of the small bowel which can develop must be treated conservatively to avoid unnecessary bowel resection.
D000328:Adult; D002566:Ceroid / Q000378:metabolism*; D003937:Diagnosis, Differential; D006801:Humans; D007413:Intestinal Mucosa / Q000378:metabolism; D007415:Intestinal Obstruction / Q000000981:diagnostic imaging*; D008297:Male; D009130:Muscle, Smooth / Q000378:metabolism; D010860:Pigments, Biological / Q000378:metabolism*; D011859:Radiography; D012595:Scleroderma, Systemic / Q000000981:diagnostic imaging; D008061:Whipple Disease / Q000150:complications* / Q000378:metabolism
D002566:Ceroid; D010860:Pigments, Biological