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D81.810 ICD-10-CM Code: Biotinidase deficiency

Billing Status: YES. This is a valid, specific, and billable ICD-10-CM reference.

Coding at a Glance

Tabular directives
3 inclusion terms · 4 Excludes1 · 1 Excludes2

Inpatient Payment Groups (MS-DRG)

Potential MS-DRG participation — not a DRG assignment.

MS-DRGs this diagnosis helps define, as principal or secondary, per the CMS ICD-10-CM/PCS MS-DRG Definitions Manual v43, Appendix B.

  • MS-DRG 642 — INBORN AND OTHER DISORDERS OF METABOLISM (MDC 10)

A diagnosis appearing in a group's logic does not by itself determine the DRG assigned to a stay; the grouper uses the full claim.

Coding instructions

Official Tabular Instructional Notes: the inclusion, exclusion and sequencing notes published for D81.810 in the official ICD-10-CM tabular list, quoted as published.

Source: CMS/CDC — ICD-10-CM Tabular ListRelease: FY2026Effective: October 1, 2025

Notes without a marker are published on D81.810 itself; “inherited from” names the category or block whose note applies here.

This page already reflects the FY2027 tabular note taking effect October 1, 2026.

Includes

Conditions the official ICD-10-CM tabular list includes under this code.

  • defects in the complement system
  • immunodeficiency disorders, except human immunodeficiency virus [HIV] disease
  • sarcoidosis

Source: inherited from D80-D89

Excludes1 — Not Coded Here

Conditions generally not reported together with this code (Excludes1) -- an error unless the two conditions are documented as unrelated to each other (ICD-10-CM Official Guidelines, Section I.A.12.a).

Excludes2 — Not Included Here

Conditions not covered by this code, but which may be reported alongside it when both are present.

Source: inherited from D80-D89

Coder workflow for D81.810

MedCoder structured workflow — derived from this code’s own official record

Before you code D81.810

  1. Check the Excludes1 note: if the documentation supports a condition named there, do not simply proceed with D81.810. Excludes1 conditions are not reported together with this code unless the record shows they are unrelated (Guidelines I.A.12.a).

    See the official tabular notes

Choose the right path

  1. Does the documentation support a condition named in D81.810’s Excludes1 note?
    Yes → Do not simply proceed: review the excluded code. Both are reported only when the record shows the two conditions are unrelated.
    No → Continue.

    ReviewE53.8, D80.0, M35.9, D71

Consider D81.810. Then confirm the code is valid for the date of service in the Verify section.

Documentation check

The provider’s diagnostic statement
Codes are assigned from the provider’s documented diagnosis, not from clinical criteria, test values or a medication list (Guidelines I.A.19).

Official instructions as workflow

  • Excludes1 — check before selecting D81.810(4 notes)

    Coding workflow: If the documentation supports a condition named in this note, do not simply proceed with D81.810: the two are not reported together. The one exception is when the record shows the two conditions are unrelated to each other.

    CompareE53.8, D80.0, M35.9, D71

    See the official tabular notes · Guidelines I.A.12.a

  • Excludes2 — not part of D81.810(1 note)

    Coding workflow: The conditions named in this note are not included in D81.810. When the record documents both, both may be reported; the note is a boundary, not a prohibition.

    CompareB20

    See the official tabular notes · Guidelines I.A.12.b

Coding decision scenarios

Pattern scenarios for this code’s structure — decision rules, not clinical cases

Documentation: Both the condition D81.810 describes and a condition named in its Excludes1 note are documented for the same encounter.

Coding question: Can both codes be reported?

Path: Review the Excludes1 note and the excluded code, and look for a provider statement on whether the two conditions are related.

Reason: Excludes1 means the two are not coded together; the exception is when the record shows the conditions are unrelated to each other (Guidelines I.A.12.a).

ReviewE53.8, D80.0, M35.9, D71

Every row is derived from this code’s own record — its title, tabular notes, 7th-character family and same-category siblings — with fixed MedCoder wording; nothing is inferred about a patient. The official notes and guideline text are in the sections each row links to, and they control.

Code Overview

Biotinidase deficiency is a billable ICD-10-CM diagnosis code (D81.810).

MedCoder summary Summary composed by MedCoder from this code's official ICD-10-CM record. The tabular instructional notes themselves appear verbatim below.

Indexed Clinical Terms (2)

Official source data — entries quoted as published, in the Index’s own lookup phrasing

Clinical term phrases from the official ICD-10-CM Index to Diseases and Injuries that map to this code. These are alphabetic-index entries shown as the Index writes them — lookup phrasing, not necessarily the wording of a final diagnosis.

Official Coding Guidelines

No excerpt in the ICD-10-CM Official Guidelines names this code specifically. Its chapter carries only this chapter-wide note:

Chapter 3: Disease of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89)

Reserved for future guideline expansion

Verify Before Coding

  • No Medicare Code Editor or MS-DRG Definitions Manual restrictions apply to this code.

From the code registry, the Medicare Code Editor, and the MS-DRG Definitions Manual. Check it against a full claim in Claim Check.

Relationships & Classification

MedCoder structured relationships — computed from published CMS and AHRQ datasets

Other codes that name D81.810 or its code family, from the CMS ICD-10-CM tabular instructional notes. Tabular-note edges are stored at the code family level that carries each note.

Referenced by 3 Excludes1 notes across 2 chapters: D72 — Other disorders of white blood cells (via D81.-), D84.81 — Immunodeficiency due to conditions classified elsewhere (via D81.-), E79 — Disorders of purine and pyrimidine metabolism (via D81.-).

These codes’ tabular lists mark this diagnosis as mutually exclusive — not reported together unless the conditions are documented as unrelated.

MS-DRG Grouper Relationships (FY2026)

Potential MS-DRG participation — not a DRG assignment.

FY2026 MS-DRG: not on the CMS CC/MCC list — as a secondary diagnosis this code does not change MS-DRG severity for that release.

Named in the grouper logic of 1 MS-DRG: DRG 642 (MDC 10).

From the CMS MS-DRG Definitions Manual (Appendices B and C). Actual DRG assignment depends on the complete claim.

Clinical classification (AHRQ CCSR):BLD008 — Immunity disorders (default); END016 — Other specified and unspecified nutritional and metabolic disorders.

Clinical Classifications Software Refined (CCSR) for ICD-10-CM Diagnoses. Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality.

Related Codes

Same clinical category (CCSR)

AHRQ's Clinical Classifications Software groups these diagnoses under the same clinical categories (Immunity disorders, Other specified and unspecified nutritional and metabolic disorders).

D81.2 — Severe combined immunodeficiency [SCID] with low or normal B-cell numbers, D81.3 — Adenosine deaminase [ADA] deficiency, D81.30 — Adenosine deaminase deficiency, unspecified, D81.31 — Severe combined immunodeficiency due to adenosine deaminase deficiency, D81.32 — Adenosine deaminase 2 deficiency, D81.39 — Other adenosine deaminase deficiency, D81.4 — Nezelof's syndrome, D81.5 — Purine nucleoside phosphorylase [PNP] deficiency, D81.6 — Major histocompatibility complex class I deficiency, D81.7 — Major histocompatibility complex class II deficiency, D81.818 — Other biotin-dependent carboxylase deficiency, D81.819 — Biotin-dependent carboxylase deficiency, unspecified, D81.82 — Activated Phosphoinositide 3-kinase Delta Syndrome [APDS], D81.89 — Other combined immunodeficiencies, D81.9 — Combined immunodeficiency, unspecified, D82.0 — Wiskott-Aldrich syndrome, D82.1 — Di George's syndrome, D82.2 — Immunodeficiency with short-limbed stature, D82.3 — Immunodeficiency following hereditary defective response to Epstein-Barr virus, D82.4 — Hyperimmunoglobulin E [IgE] syndrome, +334 more

Same Index main term, other category

The ICD-10-CM Index to Diseases and Injuries files this code under the main terms “Immunodeficiency”, “Deficiency, deficient”; these codes share that main term but sit in a different category of the Tabular List.

D75.A — Glucose-6-phosphate dehydrogenase (G6PD) deficiency without anemia (glucose-6-phosphate dehydrogenase, without anemia), D80.0 — Hereditary hypogammaglobulinemia (autosomal recessive, Swiss type), D80.1 — Nonfamilial hypogammaglobulinemia (gammaglobulin in blood), D80.2 — Selective deficiency of immunoglobulin A [IgA] (immunity, IgA), D80.3 — Selective deficiency of immunoglobulin G [IgG] subclasses (immunity, IgG), D80.4 — Selective deficiency of immunoglobulin M [IgM] (immunity, IgM), D80.5 — Immunodeficiency with increased immunoglobulin M [IgM] (X-linked, with increased IgM), D80.6 — Antibody deficiency with near-normal immunoglobulins or with hyperimmunoglobulinemia (with, hyperimmunoglobulinemia), D80.8 — Other immunodeficiencies with predominantly antibody defects (kappa-light chain), D80.9 — Immunodeficiency with predominantly antibody defects, unspecified (immunity, humoral), D82.0 — Wiskott-Aldrich syndrome (with, thrombocytopenia and eczema), D82.2 — Immunodeficiency with short-limbed stature (with, short-limbed stature), D82.3 — Immunodeficiency following hereditary defective response to Epstein-Barr virus (following hereditary defective response to Epstein-Barr virus), D82.8 — Immunodeficiency associated with other specified major defects (with, partial albinism), D82.9 — Immunodeficiency associated with major defect, unspecified (with, major defect), D83.0 — Common variable immunodeficiency with predominant abnormalities of B-cell numbers and function (common variable, with, abnormalities of B-cell numbers and function), D83.1 — Common variable immunodeficiency with predominant immunoregulatory T-cell disorders (common variable, with, immunoregulatory T-cell disorders), D83.2 — Common variable immunodeficiency with autoantibodies to B- or T-cells (common variable, with, autoantibodies to B- or T-cells), D83.8 — Other common variable immunodeficiencies (common variable, specified type NEC), D83.9 — Common variable immunodeficiency, unspecified (common variable), +178 more

Lab tests where this diagnosis supports Medicare coverage (NCD)

Medicare's National Coverage Determination (NCD) program lists this diagnosis as medical justification for these lab tests.

Alpha-Fetoprotein (AFP) Test, Gamma-glutamyl Transferase (GGT) Test

Contextual Map

Every relationship of D81.810 in one view: hierarchy, official tabular instructions in both directions, clinical classification, risk adjustment, MS-DRG participation, index terms and change history — each edge carrying the CMS source it derives from.

Run D81.810 with these 3 related codes in Claim Check

Hierarchy

Referenced by Excludes1 notes

Clinical classification (CCSR)

MS-DRG Grouper

MDC crossing

  • MDC 10 — Endocrine, Nutritional and Metabolic Diseases and Disorders[MDC crossing]: “Endocrine, Nutritional and Metabolic Diseases and Disorders — the grouper's crossing between diagnoses and procedures: a principal diagnosis sets the MDC, and same-MDC procedures move the stay to its surgical DRGs. 5,008 same-MDC procedures group here; browse them on the MDC page.”— CMS MS-DRG Definitions Manual · FY2026

Index entries

  • Deficiency, deficient, biotinidase[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026
  • Immunodeficiency, combined, biotin-dependent carboxylase, biotinidase[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2026

Nearest codes (20)

Change history (3)

Sources for this page

Codes, titles, notes, index terms and mappings on this page are transcribed from the datasets below. Relationships MedCoder computed and text MedCoder wrote are labelled where they appear.

Code, title, tabular notes and index terms Official source data
CMS/CDC ICD-10-CM FY2026 tabular list, index and tables, effective October 1, 2025 Release, file and checksum · Publisher’s page
Coding guidelines Official source data
ICD-10-CM Official Guidelines for Coding and Reporting (FY2026), quoted by section Release, file and checksum · Publisher’s page
Claim edits Official source data
CMS Definitions of Medicare Code Edits — v43.1 (April 2026) Release, file and checksum · Publisher’s page
Inpatient payment groups Official source data
CMS MS-DRG Definitions Manual (incl. Appendix B diagnosis index, Appendix C CC/MCC list, Appendix E procedure index) and IPPS Final Rule tables — v43 Release, file and checksum · Publisher’s page
Change history and date-of-service validity Official source data
CMS ICD-10-CM release addenda, ingested release by release into the change ledger Release, file and checksum · Publisher’s page
Comparisons, relationships and the contextual map MedCoder structured relationship
Computed by MedCoder from the tabular notes and tables above; every derived relationship is marked as derived where it appears
Summary and FAQ answers MedCoder explanation
Written by MedCoder to explain the sources above: drafted with AI assistance, checked by a person against the release files, and labelled as MedCoder text where it appears. Not official text.

Labels on this page: Official source data · MedCoder structured relationship · MedCoder explanation. How to read the labels · All data sources and release dates

Cite this page

Reference this page in a research guide, syllabus or article. The release is included because code content changes each year.

MedCoder.ai. "D81.810 — Biotinidase deficiency." ICD-10-CM FY2026. https://medcoder.ai/icd10/code/d81.810-biotinidase-deficiency

Change history

  • Upcoming · effective FY2027 — October 1, 2026
    Excludes2 note will be added
    human immunodeficiency virus [HIV] disease (B20)
    FY2027 changes
  • Upcoming · effective FY2027 — October 1, 2026
    Excludes1 note will be removed
    human immunodeficiency virus [HIV] disease (B20)
    FY2027 changes
  • FY2016 — October 1, 2015
    In the code set at ICD-10-CM adoption
    Biotinidase deficiency

Nearest Codes in This Family

Official ICD-10-CM classifications closest to D81.810 in its code family, with their registry titles.

View all codes in the D81 family