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    v1.0.28
    OpenPayerBilling CodesMetabolic disordersICD-10-CM e75-disorders-of-sphingolipid-metabolism
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    ICD-10-CM Code For Disorders Of Sphingolipid Metabolism And Other Lipid Storage Disorders: Group Overview

    The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group E75 includes inherited sphingolipid metabolism disorders and other lipid storage diseases such as Gaucher disease, Niemann-Pick disease, Fabry disease, and related lysosomal storage disorders that result in pathological lipid accumulation. These ICD-10-CM diagnosis codes are used to document clinical encounters involving genetic lipid storage conditions, capturing disease severity, organ involvement such as neurologic or hepatic manifestations, and clinical complexity relevant to this group. Accurate coding within the ICD-10-CM E75 group supports complete clinical documentation and appropriate reimbursement.

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    ICD-10-CM E75: Disorders Of Sphingolipid Metabolism And Other Lipid Storage Disorders Overview

    The ICD-10-CM E75 diagnosis group encompasses inherited disorders of sphingolipid metabolism and other lipid storage diseases that primarily affect lysosomal function and lipid handling. These conditions often involve neurologic, hepatic, and systemic manifestations due to abnormal lipid accumulation in tissues. Accurate coding matters for proper clinical documentation and reimbursement because it captures disease complexity, complications, and resource needs.

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    When to Use

    Use a code from this group when a patient has a confirmed diagnosis of a sphingolipid metabolism disorder or another lipid storage disease documented by clinical assessment, genetic testing, or enzyme assay. Select the most specific code that reflects the type of storage disorder, affected organs, and any documented complications to support accurate clinical and billing records.

    ICD-10-CM Codes

    26 rows

    ICD-10-CM CodeDescription
    E75.00GM2 gangliosidosis, unspecified
    E75.01Sandhoff disease
    E75.02Tay-Sachs disease
    E75.09Other GM2 gangliosidosis
    E75.10Unspecified gangliosidosis
    E75.11Mucolipidosis IV
    E75.19Other gangliosidosis
    E75.21Fabry (-Anderson) disease
    E75.22Gaucher disease
    E75.23Krabbe disease
    E75.240Niemann-Pick disease type A
    E75.241Niemann-Pick disease type B
    E75.242Niemann-Pick disease type C
    E75.243Niemann-Pick disease type D
    E75.244Niemann-Pick disease type A/B
    E75.248Other Niemann-Pick disease
    E75.249Niemann-Pick disease, unspecified
    E75.25Metachromatic leukodystrophy
    E75.26Sulfatase deficiency
    E75.27Pelizaeus-Merzbacher disease

    Showing 1–20 of 26

    DRG Codes

    3 rows

    DRG CodeDescription
    056Degenerative Nervous System Disorders with MCC
    057Degenerative Nervous System Disorders without MCC
    642Inborn and Other Disorders of Metabolism

    Typical Clinical Scenarios

    • An infant presents with progressive hypotonia, poor feeding, and developmental delay noted at two months of age; neurologic exam shows loss of milestones and seizures, and enzyme testing or genetic testing confirms deficiency of a sphingomyelinase or another enzyme causing a primary sphingolipidosis. A diagnosis from this group is assigned to specify the inherited sphingolipid storage disorder responsible for the early-onset neurodegenerative picture. Typical codes: E75.00, E75.01
    • An adult with a known chronic lipid storage disorder follows regularly in metabolic clinic for progressive peripheral neuropathy, vision changes, and recurrent bouts of pain in the extremities; ongoing genetic confirmation and assessment of multisystem involvement guide management and surveillance. A code from this group is used to document the chronic, genetically confirmed sphingolipid metabolism disorder as the primary diagnosis for longitudinal care. Typical codes: E75.21, E75.22
    • A hospitalized patient with an established sphingolipid storage disease is admitted for acute respiratory failure and aspiration pneumonia related to advanced neuromuscular weakness; the storage disorder is an important comorbidity that affects prognosis and care decisions during the acute admission. The coding captures the underlying metabolic disorder alongside the acute pulmonary diagnosis. Typical codes: E75.3, E75.4

    Related Codes

    E70-DISORDERS-OF-AROMATIC-AMINO-ACID-METABOLISM

    ICD-10-CM Code For Disorders Of Aromatic Amino-Acid Metabolism: Group Overview

    E71-DISORDERS-OF-BRANCHED-CHAIN-AMINO-ACID-METABOLISM

    ICD-10-CM Code For Disorders Of Branched Chain Amino Acid Metabolism And Fatty Acid Metabolism: Group Overview

    E72-OTHER-DISORDERS-OF-AMINO-ACID-METABOLISM

    ICD-10-CM Code For Other Disorders Of Amino-Acid Metabolism: Group Overview

    E73-LACTOSE-INTOLERANCE

    ICD-10-CM Code For Lactose Intolerance: Group Overview

    E74-OTHER-DISORDERS-OF-CARBOHYDRATE-METABOLISM

    ICD-10-CM Code For Other Disorders Of Carbohydrate Metabolism: Group Overview

    E76-DISORDERS-OF-GLYCOSAMINOGLYCAN-METABOLISM

    ICD-10-CM Code For Disorders Of Glycosaminoglycan Metabolism: Group Overview

    E77-DISORDERS-OF-GLYCOPROTEIN-METABOLISM

    ICD-10-CM Code For Disorders Of Glycoprotein Metabolism: Group Overview

    E78-DISORDERS-OF-LIPOPROTEIN-METABOLISM

    ICD-10-CM Code For Disorders Of Lipoprotein Metabolism And Other Lipidemias: Group Overview

    E79-DISORDERS-OF-PURINE-AND-PYRIMIDINE-METABOLISM

    ICD-10-CM Code For Disorders Of Purine And Pyrimidine Metabolism: Group Overview

    E80-DISORDERS-OF-PORPHYRIN-AND-BILIRUBIN-METABOLISM

    ICD-10-CM Code For Disorders Of Porphyrin And Bilirubin Metabolism: Group Overview

    Showing 1–10 of 16

    Frequently Asked Questions

    References

    1. 1.CPT® Codes and Descriptions. AAPC. Retrieved Aug 11, 2026.Maintains the CPT code set's official descriptions and guidelines, licensed from the American Medical Association, used as the definitional source for CPT codes on this page.
    2. 2.HCPCS Quarterly Update. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's quarterly release of HCPCS Level II codes and short/long descriptors, the source for HCPCS code definitions and update history.
    3. 3.ICD-10-CM Full Code List. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's complete ICD-10-CM diagnosis code list, used to cross-reference diagnosis codes associated with this procedure or service.
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