D81.810 vs E53.8

D81.810 (Biotinidase deficiency) compared with E53.8 (Deficiency of other specified B group vitamins), from the official CMS tabular data.

Summary

These codes should not be reported together because D81.810’s tabular entry lists E53.8 under Excludes1.

Official rule

What is different?

The conditions named and chapter.D81.810 is “Biotinidase deficiency”; E53.8 is “Deficiency of other specified B group vitamins”. D81.810 sits in D50-D89 — Diseases of the Blood and Blood-forming Organs and Certain Disorders Involving the Immune Mechanism (D50-D89); E53.8 in E00-E89 — Endocrine, Nutritional and Metabolic Diseases (E00-E89).

Registry fact

Can these codes be reported together?

No.D81.810’s entry carries an Excludes1 note covering E53.8: “biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8)”. Both may be reported only when the documentation shows the two conditions are unrelated to each other (ICD-10-CM Official Guidelines, Section I.A.12.a).

Official ruleGuide: Excludes1 vs Excludes2

Is there an Excludes1 relationship?

Yes.D81.810’s tabular entry: “biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8)”. The note covers E53.8.

Official ruleGuide: Excludes1 vs Excludes2

Is there an Excludes2 relationship?

None.Neither entry carries an Excludes2 note that names the other code. D81.810 carries 1 Excludes2 note and E53.8 carries 2 Excludes2 notes covering other codes; the side-by-side table quotes them.

Official rule

Is one more specific?

Not comparable.The codes sit in different categories; specificity is only comparable within one category.

Is one a parent or header code?

No.Both are billable codes, and neither contains the other.

Registry fact

Are there sequencing instructions?

None.Neither entry carries a Code First or Use Additional Code note that names the other.

Official rule

Are there other coding relationships?

None found.No laterality, encounter-phase, Table of Neoplasms, Table of Drugs and Chemicals, or history-versus-active-disease relationship links these codes.

MedCoder-derived

Official rule: a tabular instructional note or a section of the ICD-10-CM Official Guidelines, quoted as published. Registry fact: the codes’ own published attributes (titles, billable status, position in the hierarchy, code set). MedCoder-derived: a reading MedCoder computes from those facts; it is not itself a rule. How to read the labels

Can these codes be reported together?

Excludes1 — generally not reported together

Excludes1 conflict

D81.810

Biotinidase deficiency

↔

E53.8

Deficiency of other specified B group vitamins

What we found

No — do not report together.D81.810 carries an Excludes1 note covering E53.8: “biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8)”

Sole exception: when the two conditions are documented as unrelated to each other (ICD-10-CM Official Guidelines, Section I.A.12.a).

What to review

Report one of the two. Both may be reported only when the documentation shows the two conditions are unrelated to each other.

Guide: Excludes1 vs Excludes2 Check these on a claim

Why it matters

An Excludes1 note means the two conditions are not ordinarily reported together for the same encounter, because the classification treats them as mutually exclusive forms of the same condition. The documented exception is when the two conditions are unrelated to each other.

Source / rule

CMS ICD-10-CM tabular instructional notes; ICD-10-CM Official Guidelines, Section I.A.12.a

Official guidance behind these answers

Section I.A.12.a — Excludes1 · applies to: Excludes1
A type 1 Excludes note is a pure excludes note. It means “NOT CODED HERE!” An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition. An exception to the Excludes1 definition is the circumstance when the two conditions are unrelated to each other. If it is not clear whether the two conditions involving an Excludes1 note are related or not, query the provider. For example, code F45.8, Other somatoform disorders, has an Excludes1 note for "sleep related teeth grinding (G47.63)," because "teeth grinding" is an inclusion term under F45.8. Only one of these two codes should be assigned for teeth grinding. However psychogenic dysmenorrhea is also an inclusion term under F45.8, and a patient could have both this condition and sleep related teeth grinding. In this case, the two conditions are clearly unrelated to each other, and so it would be appropriate to report F45.8 and G47.63 together.
ICD-10-CM Official Guidelines FY2027

Quoted from the ICD-10-CM Official Guidelines for Coding and Reporting in effect for the release shown. The tabular notes above are the code-level instruction; these sections are the convention that says how such a note is applied. Source document (CMS PDF) · Release and checksum

Side by side

	D81.810Biotinidase deficiency	E53.8Deficiency of other specified B group vitamins
Billing status	Billable	Billable
Classification	D50-D89 — Diseases of the Blood and Blood-forming Organs and Certain Disorders Involving the Immune Mechanism (D50-D89)	E00-E89 — Endocrine, Nutritional and Metabolic Diseases (E00-E89)
Definition	Biotinidase deficiency is a billable ICD-10-CM diagnosis code (D81.810).	Deficiency of other specified B group vitamins is a billable ICD-10-CM diagnosis code (E53.8).
Includes	defects in the complement system immunodeficiency disorders, except human immunodeficiency virus [HIV] disease sarcoidosis	Biotin deficiency Cyanocobalamin deficiency Folate deficiency Folic acid deficiency
Excludes1	biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8) autosomal recessive agammaglobulinemia (Swiss type) (D80.0) autoimmune disease (systemic) NOS (M35.9) functional disorders of polymorphonuclear neutrophils (D71-)	folate deficiency anemia (D52.-) sequelae of vitamin B deficiency (E64.8)
Excludes2	human immunodeficiency virus [HIV] disease (B20)	vitamin B12 deficiency anemia (D51.-) nutritional anemias (D50-D53)

Notes are the code’s own tabular entry, quoted as published. A note that names the other code is the one the verdict above rests on.

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Derived from the official CMS ICD-10-CM tabular data (FY2027). A coding-rule comparison, not billing advice: payer-specific edits and medical-necessity policy are outside its scope. All data sources