2026 ICD-10-CM Diagnosis Code Q85.1Tuberous sclerosis

ICD-10-CM Codes›Q00-Q99›Q80-Q89›Q85

ICD-10-CM Q85.1
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

Q85.1 is a billable ICD-10-CM diagnosis code for tuberous sclerosis. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). The code is exempt from POA reporting. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified congenital anomalies.

Code Identity

ICD-10-CM Code
Q85.1
Billable Status
Yes — Valid for Submission
Code Describes
Tuberous sclerosis
Short Description
Tuberous sclerosis
Same as the full description in the CMS dataset.
Parent Code
Q85
Phakomatoses, not elsewhere classified

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ80-Q89Other congenital malformations
CategoryQ85Phakomatoses, not elsewhere classified
This CodeQ85.1Tuberous sclerosis

Present on Admission (POA)Billing

Q85.1 is exempt from POA reporting on inpatient claims to general acute care hospitals. Review other POA exempt codes.

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Angiofibroma
  • Ash leaf spot, tuberous sclerosis
  • Autosomal dominant polycystic kidney disease
  • Autosomal dominant polycystic kidney disease type 1 with tuberous sclerosis
  • Benign neoplasm of nail apparatus
  • Fibrous skin tumor of tuberous sclerosis
  • Lymphangioleiomyomatosis due to tuberous sclerosis syndrome
  • Lymphangiomyomatosis of connective tissue
  • Macule of skin
  • Periungual fibroma
  • Periungual fibroma in tuberous sclerosis
  • Pulmonary lymphangioleiomyomatosis
  • Pulmonary tuberous sclerosis
  • Tuberous sclerosis syndrome

Tabular List NotesGuidance

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

Inclusion Terms

  • Bourneville's disease
  • Epiloia

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.

    • Bourneville's disease
    • Disease, diseased
      • Bourneville (-Brissaud) (tuberous sclerosis)
    • Disease, diseased
      • Pringle's (tuberous sclerosis)
    • Epiloia
    • Neurospongioblastosis diffusa
    • Nevus
      • multiplex
    • Phakomatosis
      • Bourneville's
    • Pringle's disease(tuberous sclerosis)
    • Sclerosis, sclerotic
      • brain (generalized) (lobular)
        • tuberous
    • Sclerosis, sclerotic
      • tuberous (brain)
    • Syndrome
      • Bourneville (-Pringle)
    • Tuberous sclerosis(brain)

Clinical ClassificationClinical

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

CCSR MAL010
Other specified and unspecified congenital anomalies
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Tuberous Sclerosis

    autosomal dominant neurocutaneous syndrome classically characterized by mental retardation; epilepsy; and skin lesions (e.g., adenoma sebaceum and hypomelanotic macules). there is, however, considerable heterogeneity in the neurologic manifestations. it is also associated with cortical tuber and hamartomas formation throughout the body, especially the heart, kidneys, and eyes. mutations in two loci tsc1 and tsc2 that encode hamartin and tuberin, respectively, are associated with the disease.

  • Tuberous Sclerosis Complex 1 Protein

    an intracellular signaling and tumor suppressor protein that forms a complex with tuberous sclerosis complex 2 protein (tsc2) and other signaling factors to negatively regulate mtorc1 signaling and affect cell growth and proliferation. structurally, it interacts with tsc2 through its n-terminal, which also contains gsk-3beta phosphorylation sites and a rho-kinase activation domain. it also contains a c-terminal coiled-coil domain and ezrin-radixin-moesin (erm) domain. mutations in the tsc1 gene are associated with tuberous sclerosis.

  • Tuberous Sclerosis Complex 2 Protein

    an intracellular signaling and tumor suppressor protein that forms a complex with tuberous sclerosis complex 1 protein (tsc1) and other signaling factors to negatively regulate mtorc1 and affect cell growth and proliferation. it can also function as gtpase-activating protein (gap) for rheb gtpase to activate mtorc1 independent of its role in the complex. structurally, it interacts with tsc1 through its n-terminus, which also contains a leucine zipper and coiled-coil region. it also has multiple phosphorylation sites for different cell signaling kinases, a central coiled-coil region, a c-terminal gap domain and calmodulin binding domain. mutations in the tsc2 gene are associated with tuberous sclerosis.

  • Angiofibroma

    a benign neoplasm of fibrous tissue in which there are numerous small and large, frequently dilated, vascular channels. (stedman, 25th ed)

  • Polycystin-1|Autosomal Dominant Polycystic Kidney Disease Protein 1|PC1|Polycystic Kidney Disease-Associated Protein|Polycystin 1

    polycystin-1 (4303 aa, ~463 kda) is encoded by the human pkd1 gene. this protein may play a role in protein-protein and protein-carbohydrate interactions during kidney development.

  • Autosomal Dominant Polycystic Kidney Disease

    polycystic kidney disease inherited in an autosomal dominant pattern. symptoms usually appear at middle age and include abdominal pain, hematuria and high blood pressure. patients may develop brain aneurysms and liver cysts.

  • Autosomal Dominant Polycystic Kidney Disease Type 2

    autosomal dominant polycystic kidney disease caused by a mutation in pkd2.

  • Autosomal Dominant Polycystic Kidney Disease Type I

    autosomal dominant polycystic kidney disease caused by a mutation in pkd1.

  • Polycystic Kidney Disease, Infantile Severe, with Tuberous Sclerosis|Autosomal Dominant Polycystic Kidney Disease Type 1 with Tuberous Sclerosis|PKDTS|TSC2-PKD1 Contiguous Gene Deletion Syndrome

    an autosomal dominant condition caused by a contiguous gene deletion involving the pkd1 and tsc2 genes, encoding polycystin-1 and tuberin respectively. it is characterized by polycystic kidneys and tuberous sclerosis.

  • Polycystin-1|Autosomal Dominant Polycystic Kidney Disease Protein 1

    polycystin-1 (4303 aa, ~463 kda) is encoded by the human pkd1 gene. this protein may play a role in protein-protein and protein-carbohydrate interactions during kidney development.

  • Polycystin-1 Measurement|Autosomal Dominant Polycystic Kidney Disease 1 Protein|PC1|PKD1 Measurement|Polycystin 1, Transient Receptor Potential Channel Interacting|Polycystin-1|Polycystin-1|TRPP1

    the determination of the polycystin-1 present in a sample.

Patient EducationClinical

Tuberous Sclerosis

Tuberous sclerosis is a rare genetic disease that causes benign tumors to grow in the brain and other organs. Symptoms vary, depending on where the tumors grow. They could include:

Read the full article at MedlinePlus

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

Convert Q85.1 to ICD-9-CMHistory

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

ICD-9-CM
759.5 Tuberous sclerosis
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 Q85.1Overview

Is Q85.1 (Phakomatoses, not elsewhere classified) a billable code?

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

Is Q85.1 exempt from POA reporting?

Yes. CMS lists this code among those exempt from present on admission reporting, so hospitals do not assign a POA indicator for tuberous sclerosis on inpatient claims.

What is the ICD-9 equivalent of Q85.1?

Under the General Equivalence Mappings, tuberous sclerosis converts to ICD-9-CM 759.5 (tuberous sclerosis). 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.