ICD-10 Code E83.822 – ENPP1 deficiency causing autosomal recessive hypophosphatemic rickets type 2 (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E83.822 – ENPP1 deficiency causing autosomal recessive hypophosphatemic rickets type 2
What it is
This code identifies ENPP1 deficiency, a rare inherited disorder that disrupts phosphate balance and bone mineralization. It is used when the diagnosis specifically states autosomal recessive hypophosphatemic rickets type 2.
Clinical signs
Typical findings include rickets or osteomalacia, bone pain, deformities, and impaired growth. Clinical features vary; refer to documentation, especially when genetic confirmation or a specialist diagnosis is recorded.
When to use this code
Use E83.822 when the provider documents ENPP1 deficiency or autosomal recessive hypophosphatemic rickets type 2. It may also apply when the chart clearly links the condition to the ENPP1 gene or a confirmed inherited phosphate-wasting disorder.
Code it from the final diagnosis, problem list, or specialist note if the wording is explicit. Check documentation when only “rickets” or “hypophosphatemia” is mentioned without the specific inherited cause.
Do not use for
Do not use this code for unspecified rickets, nutritional vitamin D deficiency, or other causes of hypophosphatemia. Check documentation if the record does not clearly identify ENPP1 deficiency.
Coding tip
Assign this code only when the inherited ENPP1-related diagnosis is stated; otherwise, use the documented condition and query if needed.