Q80.9

Billable

Congenital ichthyosis, unspecified

Congenital ichthyosis, unspecified

Coding Notes

Excludes 1

Codes that cannot be used together with this code (mutual exclusion)

Excludes 2

Conditions not included here, but the patient may have both

Related Codes(6)
ICD-11 Equivalents(1)

ICD-11 Equivalents

View full mapping

Corresponding ICD-11 codes from the WHO crosswalk mapping

Also Known As / Clinical Terms(226)

SNOMED CT

Clinical Terms

  • Cutaneous syndrome with ichthyosis
  • CEDNIK (cerebral dysgenesis, neuropathy, ichthyosis, keratoderma) syndrome
  • Collodion baby
  • Ichthyosis Congenita
  • Pseudo-Addison's disease
  • Premature ovarian failure
  • PIBIDS syndrome, photosensitivity with IBIDS
  • Aptyalia
  • Ichthyosis, short stature, brachydactyly, microspherophakia syndrome
  • Atresia of bile ducts
  • Keratoderma ichthyosiform dermatosis elevated beta-glucuronidase syndrome
  • Fish scale disease
  • Salt-losing nephritis
  • Xerostomia
  • Ichthyosis, cerebellar degeneration and hepatosplenomegaly
  • Biliary atresia
  • Mutilating keratoderma of Vohwinkel
  • Passwell Goodman Siprkowski syndrome
  • LAMELLAR EXFOLIATION OF NEWBORN
  • X-linked intellectual disability, hypogonadism, ichthyosis, obesity, short stature syndrome
  • ELOVL4 (elongation of very long chain fatty acids-like 4) related neuro ichthyosis
  • MEDNIK syndrome
  • Congenital hypotrichosis
  • Congenital ichthyosis of skin
  • Congenital ichthyosis of skin (disorder)
  • NISCH (neonatal ichthyosis, sclerosing cholangitis, hypotrichosis) syndrome
  • Xeroderma, talipes and enamel defect (XTE) syndrome
  • BA - Biliary atresia
  • Hypotrichosis congenita
  • Congenital keratoderma
  • Photosensitivity with ichthyosis, brittle hair, impaired intelligence, decreased fertility and short stature syndrome
  • Severe ichthyoses
  • Vohwinkel syndrome
  • Limb reduction-ichthyosis syndrome
  • Young Hughes syndrome
  • Congenital spherophakia
  • NISCH syndrome
  • Ichthyosis congenital
  • Lentiglobus
  • Aptyalism
  • Newborn Lamellar Exfoliation
  • Congenital anomaly of lacrimal gland
  • Microspherophakia
  • Ichthyosis, oral and digital anomalies syndrome
  • Ichthyosis, intellectual disability, dwarfism, renal impairment syndrome
  • Clayton-Smith Donnai syndrome
  • Spastic tetraplegia
  • HELIX (hypohidrosis, electrolyte imbalance, lacrimal gland dysfunction, ichthyosis, xerostomia) syndrome
  • Asialia
  • POF - Premature ovarian failure
  • Ichthyosis, hypotrichosis, sclerosing cholangitis syndrome
  • Congenital hypotrichia
  • Trichothiodystrophy
  • Congenital biliary atresia
  • Fish skin
  • MEDNIK (mental retardation, enteropathy, deafness, peripheral neuropathy, ichtyosis, keratodermia) syndrome
  • Congenital ichthyosis, microcephalus, quadriplegia syndrome
  • Alacrima
  • 15q26.3 microdeletion syndrome
  • HELIX syndrome
  • Sclerosing cholangitis
  • Erythrokeratodermia variabilis Kamouraska type
  • Salt-losing nephropathy
  • Oligohidrosis
  • Mutilating keratoderma
  • Spherophakia
  • ELOVL4-related neuro ichthyosis
  • Congenital small lens
  • Vohwinkel ichthyosis syndrome
  • Congenital ichthyosis, intellectual disability, spastic quadriplegia syndrome
  • Congenital ichthyosis, microcephalus, tetraplegia syndrome
  • Osteosclerosis, ichthyosis, premature ovarian failure syndrome
  • Cerebral dysgenesis, neuropathy, ichthyosis, palmoplantar keratoderma syndrome
  • Erythrokeratodermia variabilis 3
  • Lamellar ichthyosis
  • Ichthyosis
  • Camisa disease
  • Neonatal sclerosing cholangitis, ichthyosis, hypotrichosis syndrome
  • CEDNIK syndrome
  • Intellectual disability, enteropathy, deafness, peripheral neuropathy, ichthyosis, keratoderma syndrome
  • Absent salivary secretion
  • Vohwinkel's mutilating keratoderma
  • Hypohidrosis, electrolyte imbalance, lacrimal gland dysfunction, ichthyosis, xerostomia syndrome
  • Xeroderma, talipes and enamel defect syndrome
  • Hypohydrosis
  • Newborn Lamellar Exfoliations
  • Bathing suit ichthyosis
  • Clinical xerostomia
  • Keratoderma hereditarium mutilans with ichthyosis syndrome
  • Multiple sclerosis, ichthyosis, factor VIII deficiency syndrome
  • XTE syndrome
  • Ichthyosis, follicular atrophoderma, hypotrichosis syndrome
  • Ichthyosis congenita with biliary atresia
  • Congenital ichthyosis with hypotrichosis syndrome
  • Hypohidrosis
  • Xeroderma, talipes AND enamel defect
  • Jagell, Holmgren, Hofer syndrome
  • Spastic quadriplegia
  • Congenital cataract ichthyosis syndrome
  • Autosomal dominant ichthyosis
  • Sclerosing dysplasia of bone, ichthyosis, premature ovarian failure syndrome
  • Ichthyosis, alopecia, eclabion, ectropion, intellectual disability syndrome
  • Thorn's syndrome
  • Dry mouth
  • Microphakia
  • Keratoderma hereditarium mutilans
Frequently Asked Questions
What is the ICD-10 code for congenital ichthyosis, unspecified?

The ICD-10-CM code for congenital ichthyosis, unspecified is Q80.9. The full clinical description is "Congenital ichthyosis, unspecified". Q80.9 is a billable/specific code that can be used on insurance claims and medical billing.

What does ICD-10 code Q80.9 mean?

ICD-10-CM code Q80.9 represents “Congenital ichthyosis, unspecified”. It is classified under Chapter 17: Congenital Malformations, Deformations and Chromosomal Abnormalities and is a billable/specific code that can be used on a claim.

Is Q80.9 a billable code?

Yes, Q80.9 is a billable/specific ICD-10-CM code and can be used to indicate a diagnosis on a medical claim.

What chapter is Q80.9 in?

Q80.9 is in Chapter 17: Congenital Malformations, Deformations and Chromosomal Abnormalities (codes Q00-Q99).

What codes cannot be used with Q80.9?

Q80.9 has Excludes1 notes indicating codes that cannot be used together with it, including: Refsum's disease (G60.1).

What SNOMED CT codes does Q80.9 map to?

Q80.9 maps to 45 SNOMED CT concepts: 783551005, 87715008, 253215004, 77480004, 402770002, and 40 more. SNOMED CT is a clinical terminology used in electronic health records.

What are the UMLS CUIs for Q80.9?

Q80.9 is linked to 1 UMLS Concept Unique Identifier: C0020758. The UMLS (Unified Medical Language System) integrates multiple biomedical vocabularies maintained by the U.S. National Library of Medicine.

How does Q80.9 relate to ICF functioning codes?

ICF (International Classification of Functioning, Disability and Health) codes describe how conditions like congenital ichthyosis, unspecified affect a person's functioning: body functions, activities, participation, and environmental factors. AutoICD provides ICF Core Sets for 12+ conditions and can map clinical text to ICF categories automatically. Browse the ICF directory to explore functioning codes.

What is the ICD-11 equivalent of Q80.9?

Q80.9 maps to the ICD-11 code: EC20.Y (Other specified genetic disorders of keratinisation).

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Includes SNOMED Clinical Terms® (SNOMED CT®) used by permission of SNOMED International. Includes content from the UMLS Metathesaurus, courtesy of the U.S. National Library of Medicine.