ICD-10-CM 2027
ICD-10 code for Lipidosis
From the official ICD-10-CM alphabetic index entry “Lipidosis”. Page updated September 29, 2026.
About coding Lipidosis
The ICD-10-CM code for Lipidosis is E75.6 (Lipid storage disorder, unspecified). The official index lists 7 more specific codes, so check the documentation for details such as type, cause, site or severity before settling on E75.6.
Within E75, choose E75.6 (Lipid storage disorder, unspecified) only when documentation doesn't support a more specific option: GM2 gangliosidosis (E75.0), other and unspecified gangliosidosis (E75.1), other sphingolipidosis (E75.2), sphingolipidosis, unspecified (E75.3), Neuronal ceroid lipofuscinosis (E75.4) and other lipid storage disorders (E75.5).
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 Lipidosis codes (official index)
- cerebral (infantile) (juvenile) (late) E75.4 — Neuronal ceroid lipofuscinosis
- cerebroretinal E75.4 — Neuronal ceroid lipofuscinosis
- cerebroside E75.22 — Gaucher disease
- cholesterol (cerebral) E75.5 — Other lipid storage disorders
- glycolipid E75.21 — Fabry (-Anderson) disease
- hepatosplenomegalic E78.3 — Hyperchylomicronemia
- sphingomyelin
- sulfatide E75.29 — Other sphingolipidosis
Common questions about Lipidosis ICD-10 codes
What is the ICD-10 code for Lipidosis?
- E75.6 — Lipid storage disorder, unspecified. More specific codes apply when the documentation supports them: E75.4, E75.4, E75.22, E75.5 and others below.
What is the ICD-10 code for cerebral lipidosis?
- E75.4 — Neuronal ceroid lipofuscinosis.
What is the ICD-10 code for cerebroretinal lipidosis?
- E75.4 — Neuronal ceroid lipofuscinosis.
What is the ICD-10 code for cerebroside lipidosis?
- E75.22 — Gaucher disease.
What is the ICD-10 code for cholesterol lipidosis?
- E75.5 — Other lipid storage disorders.
What is the ICD-10 code for glycolipid lipidosis?
- E75.21 — Fabry (-Anderson) disease.
What is the ICD-10 code for hepatosplenomegalic lipidosis?
- E78.3 — Hyperchylomicronemia.
Is E75.6 billable?
- Yes. E75.6 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E75.6 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E75.6 as a principal diagnosis.
Is E75.6 a CC or MCC?
- No. E75.6 is neither a CC nor an MCC under MS-DRG v44.0.