Neonatal Wells Syndrome Associated With Eosinophilic Gastroenteritis

Abstract

Background: Eosinophilic cellulitis (Wells syndrome) is an uncommon eosinophilic dermatosis of unknown pathogenesis, which signals undiagnosed infectious, malignant or systemic disease. It has been reported exceptionally in association with inflammatory bowel disease. Case report: A 7-month-old female infant, whose parents had no particular medical history, was seen for pruriginouspapulo-nodular lesions evolving since her birth. She was followed for evolutive intestinal pseudoobstruction, diagnosed as Hirschsprung disease, and was treated by discharge colostomy after an obstruction. Biological examinations showed hypereosinophilia and anaemia. Skin hispathology revealed a dense eosinophilic infiltrate, with flame figures typical of eosinophilic cellulitis. Histopathology of the resected colon showed a dense eosinophilic infiltrate throughout the bowel wall, with the presence of ganglion cells and normal nervous plexus. Discussion: This case of eosinophilic cellulitis is of particular interest because of its neonatal occurrence, nodular presentation and association with eosinophilic gastroenteritis. To our knowledge, this is the third reported association between eosinophilic cellulitis and eosinophilic gastroenteritis, which appear to have the same pathophysiology. Wells syndrome should therefore be added to the list of dermatosis associated with inflammatory bowel disease.