Congenital ichthyosis (Q80) ICD-10-CM
The Q80 code range covers congenital ichthyosis with 8 ICD-10-CM diagnosis codes. 7 of them are billable and valid for claim submission in fiscal year 2027, and the category headers group them but cannot themselves be billed.
Type 1 Excludes
A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
- Refsum's disease G60.1
Codes in the Q80 Range 8 codes · 7 billable
Clinical Terms in This Code Range
Definitions from the National Library of Medicine for conditions coded in the Q80 range.
Hyperkeratosis, Epidermolytic
A form of congenital ichthyosis inherited as an autosomal dominant trait and characterized by ERYTHRODERMA and severe hyperkeratosis. It is manifested at birth by blisters followed by the appearance of thickened, horny, verruciform scales over the entire body, but accentuated in flexural areas. Mutations in the genes that encode KERATIN-1 and KERATIN-10 have been associated with this disorder.
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 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.
About the Q80 Code Range
Congenital ichthyosis is a skin condition present at birth. It appears here among other congenital malformations.
The subdivisions distinguish named forms: Q80.0 for ichthyosis vulgaris, Q80.1 for X-linked ichthyosis, and Q80.2 for lamellar ichthyosis. Q80.3 names congenital bullous ichthyosiform erythroderma, and Q80.4 names harlequin fetus. Q80.8 is for other congenital ichthyosis; Q80.9 leaves the form unspecified.
Questions About This Page
How many billable codes are in the Q80 range?
Of the 8 codes in this range, 7 are billable and valid for claim submission from October 1, 2026 through September 30, 2027. Category header codes group them but cannot be reported on claims.
What does the Q80 range classify?
The range classifies congenital ichthyosis. Each code links to its own reference page with billing status, MS-DRG grouping, coding notes, and clinical information.
Related References
Source: CMS FY 2027 ICD-10-CM Tabular List and order file, effective October 1, 2026 through September 30, 2027.
