IPEX syndrome  From Wikipedia, the free encyclopedia
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IPEX syndrome Classification and external resources OMIM 304790
DiseasesDB 33417  IPEX (immunodysregulation polyendocrinopathy
enteropathy X-linked syndrome) is a rare disease linked to the
dysfunction of the transcription factor FOXP3, widely considered
to be the master regulator of the regulatory T cell lineage.[1]
It leads to the dysfunction of regulatory T-cells and the
subsequent autoimmunity.[2] The disorder manifests with
autoimmune enteropathy, psoriasiform or eczematous dermatitis,
nail dystrophy, autoimmune endocrinopathies (overlapping with
those seen in APECED syndrome), and autoimmune skin conditions
such as alopecia universalis and bullous pemphigoid.[2]  This
autoimmunity is called IPEX and it is the attack from the body's
own immune system against the body's own tissues and organs.
Early age onset of this disease in males causes severe
enlargement of the secondary lymphoid orga