D81.810 Biotinidase deficiency

Billable ICD-10-CM code, FY2026.

Applies from D81 Combined immunodeficiencies

Excludes1 (never code together)

• autosomal recessive agammaglobulinemia (Swiss type) (D80.0)

Applies from D81.81 Biotin-dependent carboxylase deficiency

Applicable to

• Multiple carboxylase deficiency

Excludes1 (never code together)

• biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8)

MS-DRG index

Defines the logic of DRG(s) 642 in MDC 10.

Approximate ICD-9-CM (GEMs)

• 277.6 Other deficiencies of circulating enzymes (approximate)

Code annotations containing back-references to D81.810

Back-references to D81

• Type 1 Excludes: D72 Other disorders of white blood cells ("immunity disorders (D80-D89)")
• Type 1 Excludes: D84.81 Immunodeficiency due to conditions classified elsewhere ("certain disorders involving the immune mechanism (D80-D83, D84.0, D84.1, D84.9)")
• Type 1 Excludes: E79 Disorders of purine and pyrimidine metabolism ("combined immunodeficiency disorders (D81.-)")

Diagnosis Index entries for D81.810

• Deficiency, deficient › biotinidase
• Immunodeficiency › combined › biotin-dependent carboxylase › biotinidase

Nearby codes

• D81.5 Purine nucleoside phosphorylase [PNP] deficiency
• D81.6 Major histocompatibility complex class I deficiency
• D81.7 Major histocompatibility complex class II deficiency
• D81.8 Other combined immunodeficiencies
• D81.81 Biotin-dependent carboxylase deficiency
• D81.810 Biotinidase deficiency (this code)
• 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