Catalog

X-linked immune dysregulation – polyendocrinopathy – enteropathy

No MMS code

Link to WHO Definition

X-linked immune dysregulation – polyendocrinopathy – enteropathy – http://id.who.int/icd/entity/1060287444

Immune dysregulation, Polyendocrinopahy, Enteropathy and X-linked syndrome (IPEX) is a rare disorder occurring in boys and characterised by intractable diarrhea, ichthyosiform dermatitis, early-onset insulin-dependent diabetes mellitus, thyroiditis, haemolytic anaemia, variable autoimmune phenomena and severe infections.