IPEX Syndrome

 

 

IPEX syndrome - This disease is associated with severe infant-onset severe enteropathy with villous atrophy and autoimmune disease such as diabetes, thyroid disease, or autoimmune cytopenias.  Patients typically develop an eczematous dermatitis.  IgE-mediated food allergies are common but non IgE-mediated food reactions such as food protein induced enterocolitis (FPIES) may also occur.  The management of the severe enteropathy in this disease must include avoidance of specific dietary food triggers in addition to immunosuppression.  

 

 

IPEX Syndrome