ICD-10-CM 2027
ICD-10 code for acid sphingomyelinase ASMD deficiency
From the official ICD-10-CM alphabetic index entry “Deficiency, deficient › acid sphingomyelinase (ASMD)”. Page updated September 29, 2026.
About coding acid sphingomyelinase ASMD deficiency
The ICD-10-CM code for acid sphingomyelinase ASMD deficiency is E75.249 (Niemann-Pick disease, unspecified). The official index lists 3 more specific codes, so check the documentation for details such as type, cause, site or severity before settling on E75.249.
Within E75.24, choose E75.249 (unspecified) only when documentation doesn't support a more specific option: type A (E75.240), type B (E75.241), type C (E75.242), type D (E75.243), type A/B (E75.244) and other Niemann-Pick disease (E75.248).
It is not a CC or MCC, so as a secondary diagnosis it doesn't change the MS-DRG severity level.
In MS-DRG v44.0, it is part of the grouping logic for DRG 642 (Inborn and Other Disorders of Metabolism, relative weight 1.3219), in MDC 10 (Endocrine, Nutritional and Metabolic Diseases and Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
More specific acid sphingomyelinase ASMD deficiency codes (official index)
Common questions about acid sphingomyelinase ASMD deficiency ICD-10 codes
What is the ICD-10 code for acid sphingomyelinase ASMD deficiency?
- E75.249 — Niemann-Pick disease, unspecified. More specific codes apply when the documentation supports them: E75.240, E75.244, E75.241.
Is E75.249 billable?
- Yes. E75.249 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E75.249 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E75.249 as a principal diagnosis.
Is E75.249 a CC or MCC?
- No. E75.249 is neither a CC nor an MCC under MS-DRG v44.0.