2024 ICD-10-CM Diagnosis Code Q80.9

Congenital ichthyosis, unspecified

ICD-10-CM Code:
Q80.9
ICD-10 Code for:
Congenital ichthyosis, unspecified
Is Billable?
Yes - Valid for Submission
Chronic Condition Indicator: [1]
Chronic
Code Navigator:

Code Classification

  • Congenital malformations, deformations and chromosomal abnormalities
    (Q00-Q99)
    • Other congenital malformations
      (Q80-Q89)
      • Congenital ichthyosis
        (Q80)

Q80.9 is a billable diagnosis code used to specify a medical diagnosis of congenital ichthyosis, unspecified. The code is valid during the current fiscal year for the submission of HIPAA-covered transactions from October 01, 2023 through September 30, 2024. The code is exempt from present on admission (POA) reporting for inpatient admissions to general acute care hospitals.

Unspecified diagnosis codes like Q80.9 are acceptable when clinical information is unknown or not available about a particular condition. Although a more specific code is preferable, unspecified codes should be used when such codes most accurately reflect what is known about a patient's condition. Specific diagnosis codes should not be used if not supported by the patient's medical record.

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis 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 hypotrichia
  • Congenital ichthyosis of skin
  • Congenital ichthyosis with hypotrichosis syndrome
  • Congenital ichthyosis, intellectual disability, spastic quadriplegia syndrome
  • Congenital ichthyosis, microcephalus, tetraplegia syndrome
  • Congenital keratoderma
  • Congenital keratoderma
  • Cutaneous syndrome with ichthyosis
  • 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
  • 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
  • Spastic tetraplegia
  • Spherophakia
  • Trichothiodystrophy
  • Xerostomia
  • X-linked intellectual disability, hypogonadism, ichthyosis, obesity, short stature syndrome
  • XTE syndrome

Clinical Classification

Clinical Information

  • 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.
  • 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.

Index to Diseases and Injuries References

The following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).

Present on Admission (POA)

Q80.9 is exempt from POA reporting - The Present on Admission (POA) indicator is used for diagnosis codes included in claims involving inpatient admissions to general acute care hospitals. POA indicators must be reported to CMS on each claim to facilitate the grouping of diagnoses codes into the proper Diagnostic Related Groups (DRG). CMS publishes a listing of specific diagnosis codes that are exempt from the POA reporting requirement. Review other POA exempt codes here.

CMS POA Indicator Options and Definitions

POA IndicatorReason for CodeCMS will pay the CC/MCC DRG?
YDiagnosis was present at time of inpatient admission.YES
NDiagnosis was not present at time of inpatient admission.NO
UDocumentation insufficient to determine if the condition was present at the time of inpatient admission.NO
WClinically undetermined - unable to clinically determine whether the condition was present at the time of inpatient admission.YES
1Unreported/Not used - Exempt from POA reporting. NO

Convert Q80.9 to ICD-9-CM

  • ICD-9-CM Code: 757.1 - Ichthyosis congenita
    Approximate Flag - The approximate mapping means there is not an exact match between the ICD-10 and ICD-9 codes and the mapped code is not a precise representation of the original code.

Patient Education


Skin Conditions

What does your skin do?

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:

  • Holds body fluids in, which helps prevent you from getting dehydrated
  • Keeps out harmful germs, which helps prevent infections
  • Helps you feel things like heat, cold, and pain
  • Helps control your body temperature
  • Makes vitamin D when the sun shines on it
  • Shields your body against heat and light

What problems and conditions can affect your skin?

There are many different problems and conditions which can affect your skin. Some of them can cause uncomfortable symptoms, such as itching, burning, redness, and rashes. They might also affect your appearance. Some of the more common skin conditions include:

  • Acne, which causes pimples when hair follicles under your skin get clogged up
  • Burns
  • Cuts and scrapes
  • Dandruff, flaking of the skin on your scalp (the top of your head)
  • Eczema (atopic dermatitis), which causes inflammation, redness, and irritation of the skin
  • Hives, which are red and sometimes itchy bumps on your skin
  • Insect bites
  • Psoriasis, which causes itchy, scaly red patches
  • Skin cancer
  • Skin infections

How can I keep my skin healthy?

Since your skin protects your body in many ways, it's important to try to keep your skin healthy. For example, you can:

  • Wear the right protective equipment, like gloves, long sleeves, knee and elbow pads, or helmets to protect against cuts, bumps and scrapes.
  • If you do get a cut or scrape, clean it right away with soap and warm water. Put on a bandage to protect it while it heals.
  • When you are spending time outdoors, wear long sleeves and pants and use insect repellant to prevent insect bites.
  • Prevent sunburn by covering up and using sunscreen when outdoors.
  • Wash your hands often with soap and water.
  • When you take a shower or bath, use warm (not hot) water. Use mild cleansers and wash gently (don't scrub).
  • Use moisturizers, like lotions, creams, or ointments, to prevent dry skin.

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases


[Learn More in MedlinePlus]

Ichthyosis

Ichthyosis is a group of skin disorders. with symptoms that include dry skin, itching, and redness, cracking, and scales on the skin.
[Learn More in MedlinePlus]

Code History

  • FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
  • FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
  • FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.

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.