E75.248
BillableOther Niemann-Pick disease
HCC Category Mapping
V24HCC 23 — Other Significant Endocrine and Metabolic Disorders
0.230ESRDHCC 23 — Other Significant Endocrine and Metabolic Disorders
0.000RxHCCHCC 41 — Lysosomal Storage Disorders
0.000What This Code Means
Other Niemann-Pick disease refers to rare or atypical forms of this inherited metabolic disorder that do not fit into the standard type A, B, or C classifications.
Coding Tips
- •Use this code only when the specific type of Niemann-Pick disease cannot be determined or is documented as atypical
- •Provide detailed documentation of the clinical presentation to support medical necessity and justify use of this code
Clinical Significance
Other Niemann-Pick disease captures rare or atypical presentations of Niemann-Pick disease that do not fit the standard type A, B, C, D, or A/B classifications. This may include novel mutations with unique phenotypes or cases with unusual clinical features. Accurate coding of these atypical cases is important for rare disease registries and resource allocation.
Documentation Requirements
- ✓Documented diagnosis of Niemann-Pick disease with atypical or unclassifiable features
- ✓Enzyme assay and genetic testing results
- ✓Detailed clinical presentation explaining why standard type classifications do not apply
- ✓Documentation of both neurological and visceral findings
- ✓Genetic counseling documentation and family history
Commonly Confused Codes
E75.249 — Niemann-Pick disease, unspecified: for cases where testing has not yet determined the typeE75.240-E75.244 — Specific Niemann-Pick types: use the appropriate specific code when the type is identifiedE75.22 — Gaucher disease: different lysosomal storage disorderE75.29 — Other sphingolipidosis: for non-Niemann-Pick sphingolipid disordersE88.89 — Other specified metabolic disorders: too general for documented Niemann-Pick