Villus atrophy of variable severity is present. In the typical form, abnormalities are localized mainly in the epithelium and include a disorganization of surface enterocytes with focal crowding, resembling tufts (Figure 1.5). These characteristic 'tufts' of extruding epithelium first described by Reifen et al36 are seen towards the villus tip, and may affect up to 70% of villi. The tufting process is not limited to the small intestine but also involves the colonic mucosa.11 This picture can also be observed in crypt epithelium and, in addition,
crypts often have an abnormal aspect with dilatations such as pseudocysts and abnormal regeneration with branching11 (Figure 1.6). The study of basement membrane components demonstrated an abnormal laminin and heparan sulfate proteo-glycan deposition at that level, compared to biopsy specimens from patients with celiac disease or autoimmune enteropathy.11 Relative to the controls, there was faint and irregular laminin deposition at the epithelial-lamina propria interface, while heparan sulfate proteoglycan depositions were large and lamellar, suggestive of abnormal development of basement membrane at the origin of the epithelial abnormalities. On the other hand, we observed an increased immunohisto-chemical expression of desmoglein in IED, and ultrastructural changes of desmosomes, which were increased in length and number (Figure 1.7).37
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