Clinical features:
(1) progressive malabsorption with steatorrhea
(2) fluid and electrolyte loss
(3) partial or complete failure to respond (refractory) to a gluten-free diet
(4) may require total parenteral nutrition to prevent deterioration
(5) may follow a fulminant course with renal failure and other complications
Histologic features:
(1) blunting or flattening of the villi in the small bowel
(2) an irregular band of eosinophilic, collagenous material deposited below the surface epithelium, best appreciated with a trichrome stain The appearance is the same as that seen in collagenous colitis. This may be patchy and it may take several biopsies to demonstrate it.
(3) marked subcryptal inflammation
(4) variable mucosal thinning, which may be marked
(5) occasionally concurrent collagenous colitis
Differential diagnosis: Celiac enteropathy with poor adherence to gluten-free diet
