2027 ICD-10-CM Diagnosis Code D81.810Biotinidase deficiency
ICD-10-CM Codes›D50–D89›D80-D89›D81
- Billable — Valid for Submission
- Chronic Condition
D81.810 is a billable ICD-10-CM diagnosis code for biotinidase deficiency. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 642. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Immunity disorders and Other specified and unspecified nutritional and metabolic disorders.
Code Identity
Code Classification
Approximate SynonymsGuidance
Alternate terms and clinical phrases that map to this code.
- Biotinidase deficiency
- Multiple carboxylase deficiency
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to D81.810: its own notes plus those printed at D81.81, D81, block D80-D89, and Chapter 3. A note printed at a category, block or chapter applies to every code under it.
Includes
Further defines, or gives examples of, the content of the category.
- defects in the complement system
- immunodeficiency disorders, except human immunodeficiency virus [HIV] disease
- sarcoidosis
Excludes1
Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.
- biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8)
- autosomal recessive agammaglobulinemia (Swiss type) (D80.0)
Excludes2
Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.
- human immunodeficiency virus [HIV] disease (B20)
- autoimmune disease (systemic) NOS (M35.9)
- certain conditions originating in the perinatal period (P00-P96)
- complications of pregnancy, childbirth and the puerperium (O00-O9A)
- congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
- endocrine, nutritional and metabolic diseases (E00-E88)
- human immunodeficiency virus [HIV] disease (B20)
- injury, poisoning and certain other consequences of external causes (S00-T88)
- neoplasms (C00-D49)
- symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name D81.810, its category, or a range that includes it.
Excludes1 3
These codes carry an Excludes1 note naming D81.810: they are not reported together with it, unless the two conditions are unrelated.
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
biotinidase D81.810
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
Biotinidase Deficiency
the late onset form of multiple carboxylase deficiency (deficiency of the activities of biotin-dependent enzymes propionyl-coa carboxylase, methylcrotonyl-coa carboxylase, and pyruvate carboxylase) due to a defect or deficiency in biotinidase which is essential for recycling biotin.
Patient EducationClinical
Immune System and Disorders
Your immune system is a complex network of cells, tissues, and organs. Together they help the body fight infections and other diseases.
The full article covers:
- What is the immune system?
- What are the parts of the immune system?
- How does the immune system work?
- What are the types of immunity?
- What can go wrong with the immune system?
Read the full article at MedlinePlus
Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.
Convert D81.810 to ICD-9-CMHistory
The closest ICD-9-CM equivalents under the General Equivalence Mappings.
Code HistoryHistory
Questions About D81.810Overview
What is the ICD-10 code for biotinidase deficiency?
The ICD-10-CM code for biotinidase deficiency is D81.810 (sometimes written as D81810). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
Is D81.810 (Biotin-dependent carboxylase deficiency) a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report biotinidase deficiency on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
What MS-DRG does D81.810 group to?
When biotinidase deficiency is the principal diagnosis on an inpatient stay, it groups to MS-DRG 642 (inborn and Other Disorders of Metabolism), which carries a relative weight of 1.3226. Higher weights mean higher Medicare reimbursement.
Can D81.810 be reported with E53.8?
Not as a rule. The Excludes1 note at D81.81, which applies to D81.810, lists "biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).
What is the ICD-9 equivalent of D81.810?
Under the General Equivalence Mappings, biotinidase deficiency converts to ICD-9-CM 277.6 (defic circul enzyme NEC). The mapping is approximate, so confirm the match fits the documentation.
