Also Known As
DMSDIDDM-secretory diarrhea syndromeIDDM-secretory diarrhoea syndromeIPEXX linked polyendocrinopathyX-linked autoimmunity-allergic dysregulation syndromeXLAADXPIDautoimmune enteropathy type 1autoimmunity-immunodeficiency syndrome, X-linkeddiabetes mellitus, congenital insulin-dependent, with fatal secretory diarrheadiabetes mellitus, congenital insulin-dependent, with fatal secretory diarrhoeadiarrhea, polyendocrinopathy, fatal infection syndrome, X-linkedimmune dysfunction and diarrhea syndromeimmune dysfunction and diarrhoea syndromeimmune dysregulation, polyendocrinopathy, and enteropathy X-linked syndromeimmunodeficiency, polyendocrinopathy, and enteropathy, X-linkedimmunodysregulation, polyendocrinopathy, and enteropathy, X-linkedimmunodysregulation, polyendocrinopathy, and enteropathy, X-linked, X-linked recessiveIDDM secretory diarrhea syndromeIDDM secretory diarrhoea syndromeIMMUNODYSREGULATION, polyendocrinopathy, and enteropathy, X-linkedIPEX syndromeIddm-secretory diarrhea syndromeIddm-secretory diarrhoea syndromeImmunodysregulation, polyendocrinopathy and enteropathy X-linkedautoimmunity-immunodeficiency syndrome X-linkedenteropathy, autoimmune, with hemolytic Anaemia and polyendocrinopathyenteropathy, autoimmune, with hemolytic Anemia and polyendocrinopathyimmunodeficiency, polyendocrinopathy, and enteropathy, X-linked, formerlyislets of Langerhans, absence ofpolyendocrinopathy, immune dysfunction and diarrhea X-linkedpolyendocrinopathy, immune dysfunction and diarrhoea X-linkedpolyendocrinopathy, immune dysfunction, and diarrhea, X-linked
Definition
Immunodysregulation - polyendocrinopathy - enteropathy - X-linked (IPEX) syndrome is a severe congenital systemic autoimmune disease characterized by refractory diarrhea, endocrinopathies, cutaneous involvement, and infections.