Food protein-induced enterocolitis syndrome

This code identifies a severe, non-IgE mediated food allergy primarily affecting the gastrointestinal tract, characterized by repetitive vomiting, diarrhea, and sometimes lethargy or dehydration. It is an immune response to specific food proteins, most commonly cow's milk or soy, but can also be triggered by grains or other solid foods.

Use this code for patients diagnosed with Food Protein-Induced Enterocolitis Syndrome (FPIES) based on clinical presentation and elimination/challenge testing. Documentation should clearly indicate the diagnosis of FPIES, often including the specific food allergen if identified, and rule out other causes of enterocolitis.

• Do not confuse with other food allergies (e.g., IgE-mediated anaphylaxis) or general food intolerances.
• If the specific food allergen is known, consider using an additional code from Z91.01- for allergy to food, if applicable and supported by documentation.
• Ensure documentation differentiates FPIES from other forms of enterocolitis, such as infectious or inflammatory bowel disease.