2026 ICD-10-CM Diagnosis Code H35.52Pigmentary retinal dystrophy

ICD-10-CM Codes›H00–H59›H30-H36›H35

ICD-10-CM H35.52
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

H35.52 is a billable ICD-10-CM diagnosis code for pigmentary retinal dystrophy. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 124 through 125. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Retinal and vitreous conditions.

Code Identity

ICD-10-CM Code
H35.52
Billable Status
Yes — Valid for Submission
Code Describes
Pigmentary retinal dystrophy
Short Description
Pigmentary retinal dystrophy
Same as the full description in the CMS dataset.
Parent Code
H35.5
Hereditary retinal dystrophy

Code Classification

ChapterH00–H59Diseases of the eye and adnexa
SectionH30-H36Disorders of choroid and retina
CategoryH35Other retinal disorders
This CodeH35.52Pigmentary retinal dystrophy

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Autosomal dominant retinitis pigmentosa
  • Autosomal recessive leukoencephalopathy, ischemic stroke, retinitis pigmentosa syndrome
  • Autosomal recessive posterior column ataxia and retinitis pigmentosa
  • Autosomal recessive retinitis pigmentosa
  • Body height below reference range
  • Brachydactyly, short stature, retinitis pigmentosa syndrome
  • Butterfly-shaped pigmentary macular dystrophy
  • Cholestasis with pigmentary retinopathy and cleft palate syndrome
  • Diabetes mellitus associated with genetic syndrome
  • Drusen of optic disc
  • Early onset cerebellar ataxia with retinitis pigmentosa and optic atrophy
  • Hereditary cerebellar atrophy
  • Hereditary motor and sensory neuropathy with retinitis pigmentosa
  • Hereditary retinal dystrophy primarily involving retinal pigment epithelium
  • Hypogonadotropic hypogonadism retinitis pigmentosa syndrome
  • Infantile and/or juvenile cataract
  • Juvenile cataract
  • Macular pigment deposit
  • Microphthalmia, retinitis pigmentosa, foveoschisis, optic disc drusen syndrome
  • Mitochondrial myopathy, cerebellar ataxia, pigmentary retinopathy syndrome
  • Mitochondrially encoded ATP synthase membrane subunit 6-related mitochondrial disease
  • Muscular atrophy, ataxia, retinitis pigmentosa, and diabetes mellitus
  • NARP syndrome
  • Neurological muscle weakness
  • Oculotrichodysplasia
  • Pattern dystrophy of macula
  • Polyneuropathy, hearing loss, ataxia, retinitis pigmentosa, cataract syndrome
  • Primary ciliary dyskinesia and retinitis pigmentosa syndrome
  • Progressive weakness of muscle
  • Pseudoxanthoma elasticum-like skin manifestations with retinitis pigmentosa
  • Retinal dystrophy due to systemic disorder
  • Retinal pigment deposits
  • Retinal pigment epithelial dystrophy
  • Retinitis pigmentosa
  • Retinitis pigmentosa due to systemic disease
  • Retinitis pigmentosa of bilateral eyes
  • Retinitis pigmentosa of left eye
  • Retinitis pigmentosa of right eye
  • Retinitis pigmentosa, hearing loss, premature aging, short stature, facial dysmorphism syndrome
  • Retinitis pigmentosa, intellectual disability, deafness, hypogenitalism syndrome
  • Retinitis pigmentosa, juvenile cataract, short stature, intellectual disability syndrome
  • Retinitis pigmentosa-deafness syndrome
  • Retinitis pigmentosa-deafness syndrome type 3
  • RHYNS syndrome
  • Saldino-Mainzer dysplasia
  • Spastic tetraplegia
  • Spastic tetraplegia, retinitis pigmentosa, intellectual disability syndrome
  • Tapetoretinal dystrophy
  • Uncombable hair, retinal pigmentary dystrophy, dental anomaly and brachydactyly syndrome
  • X-linked retinitis pigmentosa
  • X-linked retinitis pigmentosa heterozygote

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Albipunctate retinal dystrophy
  • Retinitis pigmentosa
  • Tapetoretinal dystrophy

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Dystrophy, dystrophia
      • retinal (hereditary)
        • pigmentary
    • Retinitis
      • pigmentosa

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR EYE005
Retinal and vitreous conditions
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Retinitis Pigmentosa

    hereditary, progressive degeneration of the retina due to death of rod photoreceptors initially and subsequent death of cone photoreceptors. it is characterized by deposition of pigment in the retina.

  • Usher Syndromes

    autosomal recessive hereditary disorders characterized by congenital sensorineural hearing loss and retinitis pigmentosa. genetically and symptomatically heterogeneous, clinical classes include type i, type ii, and type iii. their severity, age of onset of retinitis pigmentosa and the degree of vestibular dysfunction are variable.

  • Neurodegeneration with Brain Iron Accumulation 1|Brain Iron Accumulation Type I Syndrome|HARP Syndrome|Hallervorden-Spatz Disease|Hallervorden-Spatz Syndrome|Hallervorden-Spatz disease|Hypoprebetalipoproteinemia, Acanthocytosis, Retinitis Pigmentosa, and Pallidal Degeneration Syndrome|NBIA 1|NBIA1|Neuroaxonal Dystrophy, Late Infantile|PKAN Neuroaxonal Dystrophy, Juvenile-Onset|Pantothenate Kinase-Associated Neurodegeneration|Pigmentary pallidal degeneration

    a rare autosomal recessive inherited disorder caused by mutations in the pank2 gene. it is characterized by abnormal accumulation of iron in the basal ganglia. signs and symptoms include progressive motor disturbances, muscle spasm and rigidity, dysarthria, mental deterioration, and behavioral changes.

  • Pantothenate Kinase-Associated Neurodegeneration|HARP Syndrome|Hallervorden-Spatz Disease|Hypoprebetalipoproteinemia, Acanthocytosis, Retinitis Pigmentosa, and Pallidal Degeneration Syndrome|NBIA1|Neuroaxonal Dystrophy, Late Infantile|PKAN Neuroaxonal Dystrophy, Juvenile-Onset|Pigmentary pallidal degeneration

    a rare autosomal recessive inherited disorder caused by mutations in the pank2 gene. it is characterized by abnormal accumulation of iron in the basal ganglia. signs and symptoms include progressive motor disturbances, muscle spasm and rigidity, dysarthria, mental deterioration, and behavioral changes.

  • Ceramide Kinase-like Protein|Retinitis Pigmentosa 26 (Autosomal Recessive)

    ceramide kinase-like protein (558 aa, ~63 kda) is encoded by the human cerkl gene. this protein plays a role in protecting cells from apoptosis in oxidative stress conditions.

  • EYS wt Allele|C6orf178|C6orf179|C6orf180|Chromosome 6 Open Reading Frame 178 Gene|Chromosome 6 Open Reading Frame 179 Gene|Chromosome 6 Open Reading Frame 180 Gene|EGF-Like-Domain, Multiple 10 Gene|EGF-Like-Domain, Multiple 11 Gene|EGFL10|EGFL11|Eyes Shut Homolog (Drosophila) Gene|Eyes Shut Homolog wt Allele|Eyes Shut, Drosophila, Homolog of Gene|RP25|Retinitis Pigmentosa 25 (Autosomal Recessive) Gene|SPAM|Spacemaker Gene|UNQ9424/PRO34591|bA166P24.2|bA307F22.3|bA74E24.1|dJ1018A4.2|dJ22I17.2|dJ303F19.1

    human eys wild-type allele is located in the vicinity of 6q12 and is approximately 1987 kb in length. this allele, which encodes protein eyes shut homolog, is involved in retinal function. mutation of the gene is associated with autosomal recessive retinitis pigmentosa 25.

  • OFD1 wt Allele|71-7A|CXorf5|Chromosome X Open Reading Frame 5 Gene|JBTS10|MGC117039|MGC117040|Oral-Facial-Digital Syndrome 1 wt Allele|Retinitis Pigmentosa 23 (X-Linked Recessive) Gene|SGBS2

    human ofd1 wild-type allele is located in the vicinity of xp22 and is approximately 35 kb in length. this allele, which encodes oral-facial-digital syndrome 1, may be involved in the mediation of embryonic development. mutations in the gene are associated with both oral-facial-digital syndrome type i and simpson-golabi-behmel syndrome type 2.

  • POLR1D wt Allele|AC19|MGC9850|POLR1C|Polymerase (RNA) I Polypeptide D, 16kDa Gene|Polymerase (RNA) I Subunit D Gene|Polymerase I, RNA, Subunit D Gene|RNA Polymerase A, 16-kD, Mouse, Homolog of Gene|RNA Polymerase I and III Subunit D wt Allele|RPA16|RPA9|RPAC2|RPC16|RPO1-3|Retinitis Pigmentosa 25 (Autosomal Recessive) Gene|TCS2

    human polr1d wild-type allele is located in the vicinity of 13q12.2 and is approximately 123 kb in length. this allele, which encodes dna-directed rna polymerases i and iii subunit rpac2 protein, is involved in rna polymerase i- and iii-dependent transcription of rrna and other small rnas. mutation of the gene is associated with treacher collins syndrome 2.

  • Retinitis Pigmentosa

    a rare inherited retinal dystrophy disorder characterized by spots of black bone-spicule pigmentation of the retinal pigment epithelium. it is manifested with decreased vision in low light or in the night, followed by decreased peripheral vision, and, eventual decreased central vision. it may lead to blindness.

  • RHO wt Allele|CSNBAD1|OPN2|Opsin 2, Rod Pigment Gene|RP4|Retinitis Pigmentosa 4, Autosomal Dominant Gene|Rhodopsin wt Allele

    human rho wild-type allele is located in the vicinity of 3q22.1 and is approximately 7 kb in length. this allele, which encodes rhodopsin protein, is involved in photoreceptor cell activity and maintenance. mutation of the gene is associated with congenital stationary night blindness, retinitis pigmentosa 4, and retinitis punctata albescens.

  • RP2 Gene|RP2|RP2|Retinitis Pigmentosa 2 (X-Linked Recessive) Gene

    this gene plays a role in development.

  • RP2 wt Allele|DELXp11.3|NME10|Retinitis Pigmentosa 2 (X-Linked Recessive) wt Allele|TBCCD2|XRP2

    human rp2 wild-type allele is located within xp11.4-xp11.21 and is approximately 45 kb in length. this allele, which encodes protein xrp2, plays a role in photoreceptor development. mutations in this gene are associated with x-linked mental retardation with retinitis pigmentosa.

  • CERKL wt Allele|Ceramide Kinase Like wt Allele|Ceramide Kinase-like Gene|RP26|Retinitis Pigmentosa 26 (Autosomal Recessive) Gene|Retinitis Pigmentosa 26 Gene

    human cerkl wild-type allele is located in the vicinity of 2q31.3 and is approximately 144 kb in length. this allele, which encodes ceramide kinase-like protein, is involved in protecting cells from apoptosis in oxidative stress conditions.

  • MT-ATP6 wt Allele|ATP Synthase 6 Gene|ATP6|ATPASE-6|ATPase-6|ATPase6|Complex V, ATP Synthase, Subunit ATPase6 Gene|MTATP6|Mitochondrially Encoded ATP Synthase Gene|Mitochondrially Encoded ATP Synthase Membrane Subunit 6 wt Allele|Mitochondrially Encoded ATP Synthase Membrane Subunit A Gene|Spicular Retinitis Pigmentosa with Dementia, Seizures, Ataxia, Proximal Muscle Weakness and Sensory Deficit Gene|Su6m

    human mt-atp6 wild-type allele is located within the circular mitochondrial (mt) chromosome and is approximately 681 bases in length. this allele, which encodes atp synthase subunit a protein, plays a role in proton-transport and atp synthesis. mutation of the gene is associated with neuropathy, ataxia, and retinitis pigmentosa (narp); leber hereditary optic neuropathy (lhon); leigh syndrome (ls); mitochondrial infantile bilateral striatal necrosis (mibsn); myopathy, lactic acidosis, and sideroblastic anemia 3 (mlasa3); adult-onset ataxia and polyneuropathy (apao); infantile hypertrophic cardiomyopathy (cmhi); and mitochondrial complex v (atp synthase) deficiency mitochondrial type 1 (mc5dm1).

  • Polyneuropathy, Hearing Loss, Ataxia, Retinitis Pigmentosa, and Cataract|PHARC

    an autosomal recessive condition caused by mutation(s) in the abhd12 gene, encoding lysophosphatidylserine lipase abhd12. it is characterized by polyneuropathy, hearing loss, ataxia, retinitis pigmentosa and cataract.

Patient EducationClinical

Retinal Disorders

The retina is a layer of tissue in the back of your eye that senses light and sends images to your brain. In the center of this nerve tissue is the macula. It provides the sharp, central vision needed for reading, driving and seeing fine detail.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert H35.52 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
362.74 Pigment retina dystrophy
Exact Match The mapping is direct, with no qualifiers.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About H35.52Overview

Is H35.52 (Hereditary retinal dystrophy) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report pigmentary retinal dystrophy on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does H35.52 group to?

When pigmentary retinal dystrophy is the principal diagnosis on an inpatient stay, it groups to MS-DRG 124, 125, with relative weights from 0.7678 to 1.3231 depending on complications. Higher weights mean higher Medicare reimbursement.

What is the ICD-9 equivalent of H35.52?

Under the General Equivalence Mappings, pigmentary retinal dystrophy converts to ICD-9-CM 362.74 (pigment retina dystrophy). The mapping is a direct match.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.