Tufting enteropathy is a chronic watery diarrhea syndrome presenting in the first few months of life. Rarely, the disease develops in older patients. Its etiology is unknown. The disease is thought to have a genetic basis since it tends to cluster in certain families (550). Alterations suggestive of abnormal cell–cell and cell–matrix interactions are present. These include an abnormal distribution of α2β1 integrin along the crypt–villus axis, increased immunohistochemical expression of desmoglein, and ultrastructural alterations in the desmosomes (551,552).