Q80.9
BillableCongenital 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 mappingCorresponding ICD-11 codes from the WHO crosswalk mapping
Also Known As / Clinical Terms(226)
SNOMED CT
- Congenital spherophakia4465002
- Spherophakia4465002
- Congenital keratoderma6874009
- XTE syndrome7037003
- Xeroderma, talipes AND enamel defect7037003
- Xeroderma, talipes and enamel defect (XTE) syndrome7037003
- Xeroderma, talipes and enamel defect syndrome7037003
- Congenital ichthyosis13059002
- Congenital ichthyosis of skin13059002
- Fish scale disease13059002
- Fish skin13059002
- Ichthyosis congenita13059002
- Keratoderma hereditarium mutilans24559001
- Mutilating keratoderma24559001
- Mutilating keratoderma of Vohwinkel24559001
- Vohwinkel syndrome24559001
- Vohwinkel's mutilating keratoderma24559001
- Congenital small lens35272001
- Microphakia35272001
- Congenital anomaly of lacrimal gland41620007
- Hypohidrosis45004005
- Hypohydrosis45004005
- Oligohidrosis45004005
- Congenital hypotrichia56558005
- Congenital hypotrichosis56558005
- Hypotrichosis congenita56558005
- Limb reduction-ichthyosis syndrome68551007
- Atresia of bile ducts77480004
- BA - Biliary atresia77480004
- Biliary atresia77480004
- Congenital biliary atresia77480004
- Absent salivary secretion87715008
- Aptyalia87715008
- Aptyalism87715008
- Asialia87715008
- Clinical xerostomia87715008
- Dry mouth87715008
- Xerostomia87715008
- Pseudo-Addison's disease91003006
- Salt-losing nephritis91003006
- Salt-losing nephropathy91003006
- Thorn's syndrome91003006
- Spastic quadriplegia192965001
- Spastic tetraplegia192965001
- Collodion baby205550003
- Lamellar ichthyosis205550003
- Ichthyosis congenita with biliary atresia235916001
- Sclerosing cholangitis235917005
- POF - Premature ovarian failure237788002
- Premature ovarian failure237788002
- Cutaneous syndrome with ichthyosis239057002
- Alacrima253215004
- Severe ichthyoses254159008
- Autosomal dominant ichthyosis402770002
- Ichthyosis, cerebellar degeneration and hepatosplenomegaly403779009
- PIBIDS syndrome, photosensitivity with IBIDS403781006
- Photosensitivity with ichthyosis, brittle hair, impaired intelligence, decreased fertility and short stature syndrome403781006
- Microspherophakia416671000
- Lentiglobus419281007
- Camisa disease717183001
- Keratoderma hereditarium mutilans with ichthyosis syndrome717183001
- Keratoderma ichthyosiform dermatosis elevated beta-glucuronidase syndrome717183001
- Vohwinkel ichthyosis syndrome717183001
- Erythrokeratodermia variabilis 3722035007
- Erythrokeratodermia variabilis Kamouraska type722035007
- Intellectual disability, enteropathy, deafness, peripheral neuropathy, ichthyosis, keratoderma syndrome722035007
- MEDNIK (mental retardation, enteropathy, deafness, peripheral neuropathy, ichtyosis, keratodermia) syndrome722035007
- MEDNIK syndrome722035007
- Osteosclerosis, ichthyosis, premature ovarian failure syndrome722114007
- Sclerosing dysplasia of bone, ichthyosis, premature ovarian failure syndrome722114007
- CEDNIK (cerebral dysgenesis, neuropathy, ichthyosis, keratoderma) syndrome722385008
- CEDNIK syndrome722385008
- Cerebral dysgenesis, neuropathy, ichthyosis, palmoplantar keratoderma syndrome722385008
- Trichothiodystrophy723551003
- Congenital ichthyosis with hypotrichosis syndrome724277002
- Ichthyosis, follicular atrophoderma, hypotrichosis syndrome724277002
- Ichthyosis, hypotrichosis, sclerosing cholangitis syndrome724278007
- NISCH (neonatal ichthyosis, sclerosing cholangitis, hypotrichosis) syndrome724278007
- NISCH syndrome724278007
- Neonatal sclerosing cholangitis, ichthyosis, hypotrichosis syndrome724278007
- Bathing suit ichthyosis725588002
- Clayton-Smith Donnai syndrome732950006
- Ichthyosis, oral and digital anomalies syndrome732950006
- Congenital cataract ichthyosis syndrome732952003
- Multiple sclerosis, ichthyosis, factor VIII deficiency syndrome733028000
- Ichthyosis, intellectual disability, dwarfism, renal impairment syndrome733097003
- Passwell Goodman Siprkowski syndrome733097003
- Ichthyosis, alopecia, eclabion, ectropion, intellectual disability syndrome763404001
- Jagell, Holmgren, Hofer syndrome763404001
- X-linked intellectual disability, hypogonadism, ichthyosis, obesity, short stature syndrome765471005
- Young Hughes syndrome765471005
- Ichthyosis782957005
- 15q26.3 microdeletion syndrome783551005
- Ichthyosis, short stature, brachydactyly, microspherophakia syndrome783551005
- Congenital ichthyosis, microcephalus, quadriplegia syndrome1197059004
- Congenital ichthyosis, microcephalus, tetraplegia syndrome1197059004
- Congenital ichthyosis, intellectual disability, spastic quadriplegia syndrome1208936008
- ELOVL4 (elongation of very long chain fatty acids-like 4) related neuro ichthyosis1208936008
- ELOVL4-related neuro ichthyosis1208936008
- HELIX (hypohidrosis, electrolyte imbalance, lacrimal gland dysfunction, ichthyosis, xerostomia) syndrome1217380005
- HELIX syndrome1217380005
- Hypohidrosis, electrolyte imbalance, lacrimal gland dysfunction, ichthyosis, xerostomia syndrome1217380005
UMLS
- Congenital ichthyosisC0020758
- Congenital ichthyosis of skinC0020758
- Congenital ichthyosis of skin (disorder)C0020758
- Congenital ichthyosis, unspecifiedC0020758
- Fish scale diseaseC0020758
- Fish skinC0020758
- ICHTHYOSIS CONGENITAC0020758
- Ichthyosis CongenitaC0020758
- Ichthyosis congenitaC0020758
- Ichthyosis congenitalC0020758
- LAMELLAR EXFOLIATION OF NEWBORNC0020758
- Lamellar Exfoliation of NewbornC0020758
- Newborn Lamellar ExfoliationC0020758
- Newborn Lamellar ExfoliationsC0020758
- congenital ichthyosisC0020758
- fish skinC0020758
- ichthyosisC0020758
- ichthyosis congenitaC0020758
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.