2026 ICD-10-CM Diagnosis Code Q80.9Congenital ichthyosis, unspecified

ICD-10-CM Codes›Q00-Q99›Q80-Q89›Q80

ICD-10-CM Q80.9
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

Q80.9 is a billable ICD-10-CM diagnosis code for congenital ichthyosis, unspecified. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 606 through 607. 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
Q80.9
Billable Status
Yes — Valid for Submission
Code Describes
Congenital ichthyosis, unspecified
Short Description
Congenital ichthyosis, unspecified
Same as the full description in the CMS dataset.
Parent Code
Q80
Congenital ichthyosis

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ80-Q89Other congenital malformations
CategoryQ80Congenital ichthyosis
This CodeQ80.9Congenital ichthyosis, unspecified

Present on Admission (POA)Billing

Q80.9 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.

  • Alacrima
  • Autosomal dominant ichthyosis
  • Bathing suit ichthyosis
  • CEDNIK syndrome
  • Congenital anomaly of lacrimal gland
  • Congenital biliary atresia
  • Congenital cataract ichthyosis syndrome
  • Congenital hypotrichia
  • Congenital ichthyosis of skin
  • Congenital ichthyosis with hypotrichosis syndrome
  • Congenital ichthyosis, intellectual disability, spastic quadriplegia syndrome
  • Congenital ichthyosis, microcephalus, tetraplegia syndrome
  • Congenital keratoderma
  • Cutaneous syndrome with ichthyosis
  • HELIX syndrome
  • Hypohidrosis
  • Ichthyosis
  • Ichthyosis congenita with biliary atresia
  • Ichthyosis, alopecia, eclabion, ectropion, intellectual disability syndrome
  • Ichthyosis, cerebellar degeneration and hepatosplenomegaly
  • Ichthyosis, intellectual disability, dwarfism, renal impairment syndrome
  • Ichthyosis, oral and digital anomalies syndrome
  • Ichthyosis, short stature, brachydactyly, microspherophakia syndrome
  • Keratoderma hereditarium mutilans with ichthyosis syndrome
  • Lamellar ichthyosis
  • Lentiglobus
  • Limb reduction-ichthyosis syndrome
  • MEDNIK syndrome
  • Microphakia
  • Microspherophakia
  • Multiple sclerosis, ichthyosis, factor VIII deficiency syndrome
  • Mutilating keratoderma
  • Neonatal sclerosing cholangitis, ichthyosis, hypotrichosis syndrome
  • Photosensitivity with ichthyosis, brittle hair, impaired intelligence, decreased fertility and short stature syndrome
  • Premature ovarian failure
  • Salt-losing nephropathy
  • Sclerosing cholangitis
  • Sclerosing dysplasia of bone, ichthyosis, premature ovarian failure syndrome
  • Severe ichthyoses
  • Spastic tetraplegia
  • Spherophakia
  • Trichothiodystrophy
  • Xerostomia
  • X-linked intellectual disability, hypogonadism, ichthyosis, obesity, short stature syndrome
  • XTE syndrome

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.

    • Alligator skin disease
    • Disease, diseased
      • alligator-skin
    • Disease, diseased
      • fish-skin
    • Ichthyosis(congenital)

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

  • Hypohidrosis

    abnormally diminished or absent perspiration. both generalized and segmented (reduced or absent sweating in circumscribed locations) forms of the disease are usually associated with other underlying conditions.

  • Ichthyosis

    any of several generalized skin disorders characterized by dryness, roughness, and scaliness, due to hypertrophy of the stratum corneum epidermis. most are genetic, but some are acquired, developing in association with other systemic disease or genetic syndrome.

  • Ichthyosis Bullosa of Siemens

    an autosomal dominant form of ichthyosis characterized by generalized reddening of the skin (erythema) and widespread blistering. the disease shows similar, but somewhat milder, clinical and histopathological findings to those in hyperkeratosis, epidermolytic and is associated with the gene that encodes keratin-2a.

  • Ichthyosis Vulgaris

    most common form of ichthyosis characterized by prominent scaling especially on the exterior surfaces of the extremities. it is inherited as an autosomal dominant trait.

  • Ichthyosis, Lamellar

    a chronic, congenital ichthyosis inherited as an autosomal recessive trait. infants are usually born encased in a collodion membrane which sheds within a few weeks. scaling is generalized and marked with grayish-brown quadrilateral scales, adherent at their centers and free at the edges. in some cases, scales are so thick that they resemble armored plate.

  • Ichthyosis, X-Linked

    chronic form of ichthyosis that is inherited as a sex-linked recessive trait carried on the x-chromosome and transmitted to the male offspring. it is characterized by severe scaling, especially on the extremities, and is associated with steroid sulfatase deficiency.

  • Sjogren-Larsson Syndrome

    an autosomal recessive neurocutaneous disorder characterized by severe ichthyosis mental retardation; spastic paraplegia; and congenital ichthyosis. it is caused by mutation of gene encoding microsomal fatty aldehyde dehydrogenase leading to defect in fatty alcohol metabolism.

  • Trichothiodystrophy Syndromes

    autosomal recessive neuroectodermal disorders characterized by brittle sulfur-deficient hair associated with impaired intellect, decreased fertility, and short stature. it may include nail dystrophy, ichthyosis, and photosensitivity correlated with a nucleotide excision repair defect. all individuals with this disorder have a deficiency of cysteine-rich keratin-associated proteins found in the interfilamentous matrix. photosensitive trichothiodystrophy can be caused by mutation in at least 2 separate genes: ercc2 protein gene and the related ercc3. nonphotosensitive trichothiodystrophy can be caused by mutation in the ttdn1 gene.

  • Xerostomia

    decreased salivary flow.

  • Spherophakia

    a congenital disorder of the eye where the lens is abnormally small and spherical.

  • Weill-Marchesani Syndrome 1|Congenital Mesodermal Dysmorphodystrophy|Spherophakia-Brachymorphia Syndrome|Spherophakia-brachymorphia syndrome|Weill-Marchesani, Autosomal Recessive

    an autosomal recessive subtype of weill-marchesani syndrome caused by mutations in the adamts10 gene, encoding a disintegrin and metalloproteinase with thrombospondin motifs 10.

  • Grade 2 Hypohidrosis, CTCAE|Grade 2 Hypohidrosis

    symptomatic; limiting instrumental adl or mild/moderate impact on age-appropriate normal daily activity (pediatric)

  • Grade 3 Hypohidrosis, CTCAE|Grade 3 Hypohidrosis

    increase in body temperature; limiting self-care adl or severe impact on age-appropriate normal daily activity (pediatric)

  • Grade 4 Hypohidrosis, CTCAE|Grade 4 Hypohidrosis

    life-threatening consequences; urgent intervention indicated

  • X-Linked Ichthyosis|Ichthyosis, X-linked|Steryl-sulfate sulfohydrolase deficiency|X-linked placental steryl-sulfatase deficiency

    the second most common form of ichthyosis. it is an x-linked inherited disorder with mild skin manifestations. the skin changes appear at birth and include keratinization and scaling.

  • ABCA12 wt Allele|ABC12|ARCI4A|ARCI4B|ATP Binding Cassette Subfamily A Member 12 wt Allele|ATP-Binding Cassette, Sub-Family A (ABC1), Member 12 Gene|ATP-Binding Cassette, Subfamily A, Member 12 Gene|DKFZP434G232|ICR2B|Ichthyosis Congenita II, Lamellar Ichthyosis B Gene|LI2

    human abca12 wild-type allele is located in the vicinity of 2q35 and is approximately 207 kb in length. this allele, which encodes glucosylceramide transporter abca12 protein, plays a role in both the membrane localization of glucosylceramide and other lipids in lamellar granules and in cholesterol transport. mutation of the gene is associated with autosomal recessive congenital ichthyosis (arci) types 4a and 4b (harlequin).

  • Lamellar Ichthyosis

    a very rare, autosomal recessive inherited skin disorder present at birth. it is characterized by the presence of a transparent membrane encasing the newborn. this membrane sheds in about two weeks after birth to reveal generalized scaling and skin erythema.

  • Grade 2 Hypohidrosis, CTCAE|Grade 2 Hypohidrosis

    symptomatic; limiting instrumental adl

  • Grade 3 Hypohidrosis, CTCAE|Grade 3 Hypohidrosis

    increase in body temperature; limiting self care adl

  • Grade 4 Hypohidrosis, CTCAE|Grade 4 Hypohidrosis

    heat stroke

  • Grade 5 Hypohidrosis, CTCAE|Grade 5 Hypohidrosis

    death

  • Hypohidrosis

    reduced sweating. causes include burns, dehydration, radiation, and leprosy.

  • Hypohidrosis, CTCAE|Hypohidrosis|Hypohidrosis

    a disorder characterized by reduced sweating.

  • Autosomal Recessive Congenital Ichthyosis 2|ARCI2

    an autosomal recessive condition caused by mutation(s) in the alox12b gene, encoding arachidonate 12-lipoxygenase, 12r-type. it is characterized by dry, thickened, scaly skin.

  • Epidermolytic Ichthyosis|BCIE|Bullous Congenital Ichthyosiform Erythroderma|Epidermolytic Hyperkeratosis

    an autosomal dominant inherited skin disorder caused by mutations in the krt1 and krt10 genes. it is manifested at birth and is characterized by generalized erythema, skin blisters and skin fragility.

  • Harlequin Ichthyosis

    a very rare and usually lethal autosomal recessive inherited disorder of the skin caused by mutations in the abca12 gene. it is characterized by the presence of hard and thick skin. there are diamond-like plates formed in the skin which are separated by fissures.

  • Ichthyosis

    a group of inherited or acquired skin disorders characterized by a dry, thickened, and scaly skin. the skin changes range from mild to severe.

  • Ichthyosis Acquisita|Acquired Ichthyosis|Acquired Ichthyosis

    a non-hereditary form of ichthyosis characterized by plate-like scales on the legs, arms and occasionally the torso.

  • Ichthyosis Bullosa of Siemens

    a rare autosomal dominant inherited form of ichthyosis. it is characterized by the presence of an erythematous skin with blisters at birth. the skin subsequently becomes dry, flaky and hyperkeratotic.

  • Ichthyosis Vulgaris

    the most common form of ichthyosis. it is an autosomal dominant inherited or acquired disorder characterized by scaling and desquamation of the skin.

  • X-Linked Ichthyosis|Ichthyosis, X-linked

    the second most common form of ichthyosis. it is an x-linked inherited disorder with mild skin manifestations. the skin changes appear at birth and include keratinization and scaling.

Patient EducationClinical

Skin Conditions

Your skin is your body's largest organ. It covers the entire outside of your body. There are many ways that your skin protects your body and helps keep you healthy. For example, it:

The full article covers:

  • What does your skin do?
  • What problems and conditions can affect your skin?
  • How can I keep my skin healthy?

Read the full article at MedlinePlus

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

Convert Q80.9 to ICD-9-CMHistory

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

ICD-9-CM
757.1 Ichthyosis congenita
Approximate The match is approximate rather than exact.

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 Q80.9Overview

Is Q80.9 (Congenital ichthyosis) a billable code?

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

What MS-DRG does Q80.9 group to?

When congenital ichthyosis, unspecified is the principal diagnosis on an inpatient stay, it groups to MS-DRG 606, 607, with relative weights from 0.9064 to 1.5132 depending on complications. Higher weights mean higher Medicare reimbursement.

Is Q80.9 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 congenital ichthyosis, unspecified on inpatient claims.

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

Under the General Equivalence Mappings, congenital ichthyosis, unspecified converts to ICD-9-CM 757.1 (ichthyosis congenita). The mapping is approximate, so confirm the match fits the documentation.

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.