Enteropathy with loss of enteroendocrine and paneth cells in a patient with immune dysregulation: a case of adult autoimmune enteropathy
H Al Khalidi, G Kandel, CJ Streutker - Human pathology, 2006 - Elsevier
Autoimmune enteropathy (AIE) is a relatively rare condition found most frequently in
children. It presents with persistent watery diarrhea and malabsorption and may require total
parenteral nutrition for nutritional support. Rare cases have been reported in adults. On
histology, the small intestinal villi are flattened but lack the intraepithelial lymphocytosis of
celiac disease. In children and rarely in adults, some cases are linked to the IPEX syndrome
(Immune dysregulation, Polyendocrinopathy, Enteropathy, X-linked inheritance). We report a …
children. It presents with persistent watery diarrhea and malabsorption and may require total
parenteral nutrition for nutritional support. Rare cases have been reported in adults. On
histology, the small intestinal villi are flattened but lack the intraepithelial lymphocytosis of
celiac disease. In children and rarely in adults, some cases are linked to the IPEX syndrome
(Immune dysregulation, Polyendocrinopathy, Enteropathy, X-linked inheritance). We report a …