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    OpenPayerBilling CodesOther congenital malformationsICD-10-CM q80-congenital-ichthyosis
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    ICD-10-CM Code For Congenital Ichthyosis: Group Overview

    The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group Q80 includes inherited disorders of keratinization and cornification such as lamellar ichthyosis, congenital ichthyosiform erythroderma, and other specified and unspecified congenital ichthyoses that present with generalized scaling and skin barrier dysfunction. These ICD-10-CM diagnosis codes are used to document clinical scenarios ranging from mild generalized scaling to severe, life-threatening skin involvement and to capture disease severity, associated systemic features, or complications. Accurate coding within the ICD-10-CM Q80 group supports proper claim adjudication, complete clinical documentation, and appropriate reimbursement.

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    ICD-10-CM Q80: Congenital Ichthyosis Overview

    The ICD-10-CM Q80 group represents inherited disorders of cornification characterized by widespread scaling and abnormal skin barrier function. These diagnoses target the integumentary system and include various forms of congenital ichthyosis with differing severity and associated features. Accurate coding matters for complete clinical documentation and supports appropriate reimbursement by reflecting disease complexity.

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    When to Use

    Use a code from this group when documenting a congenital ichthyosis diagnosis confirmed by clinical evaluation, genetic testing, or specialist assessment. Select the most specific code available to reflect the form and severity of ichthyosis and any documented complications or associated findings.

    ICD-10-CM Codes

    7 rows

    ICD-10-CM CodeDescription
    Q80.0Ichthyosis vulgaris
    Q80.1X-linked ichthyosis
    Q80.2Lamellar ichthyosis
    Q80.3Congenital bullous ichthyosiform erythroderma
    Q80.4Harlequin fetus
    Q80.8Other congenital ichthyosis
    Q80.9Congenital ichthyosis, unspecified

    DRG Codes

    2 rows

    DRG CodeDescription
    606Minor Skin Disorders with MCC
    607Minor Skin Disorders without MCC

    Typical Clinical Scenarios

    • A newborn is evaluated in the newborn nursery for generalized, thick, plate-like scaling with ectropion and tight skin folds noted at birth; neonatal dermatology and genetics are consulted for suspected hereditary ichthyosis with systemic risks such as dehydration and infection. The infant is admitted for monitoring of fluid balance, thermal regulation, and initiation of skin care; a congenital ichthyosis diagnosis from this group is assigned based on clinical exam and family history. Typical codes: Q80.0, Q80.2
    • An adult with a lifelong history of dry, scaly skin presents to dermatology because of progressive widespread hyperkeratosis and recurrent fissuring causing pain and secondary bacterial superinfection; prior records indicate a congenital onset that has required chronic topical therapy and periodic systemic treatment. The visit documents the chronic, inherited nature of the disorder and current complications, so a code from this congenital ichthyosis group is used to capture the underlying inherited skin condition as the primary diagnosis. Typical codes: Q80.1, Q80.3
    • A pediatric patient with known congenital ichthyosis is brought to the emergency department with fever, increased erythema and purulence around fissures, and signs of systemic infection requiring IV antibiotics and wound care; the acute presentation represents an infectious complication of the underlying congenital skin barrier defect. The ED and admitting teams code the inherited ichthyosis as the underlying condition contributing to the complication, alongside the acute infectious diagnosis, to reflect the causal relationship. Typical codes: Q80.4, Q80.8

    Related Codes

    Q81-EPIDERMOLYSIS-BULLOSA

    ICD-10-CM Code For Epidermolysis Bullosa: Group Overview

    Q82-OTHER-CONGENITAL-MALFORMATIONS-OF-SKIN

    ICD-10-CM Code For Other Congenital Malformations Of Skin: Group Overview

    Q84-OTHER-CONGENITAL-MALFORMATIONS-OF-INTEGUMENT

    ICD-10-CM Code For Other Congenital Malformations Of Integument: Group Overview

    Q85-PHAKOMATOSES-NOT-ELSEWHERE-CLASSIFIED

    ICD-10-CM Code For Phakomatoses Not Elsewhere Classified: Group Overview

    Q86-CONGENITAL-MALFORMATION-SYNDROMES

    ICD-10-CM Code For Congenital Malformation Syndromes Due To Known Exogenous Causes: Group Overview

    Q87-OTHER-SPECIFIED-CONGENITAL-MALFORMATION-SYNDROMES

    ICD-10-CM Code For Other Specified Congenital Malformation Syndromes Affecting Multiple Systems: Group Overview

    Q89-OTHER-CONGENITAL-MALFORMATIONS

    ICD-10-CM Code For Other Congenital Malformations Not Elsewhere Classified: Group Overview

    Frequently Asked Questions

    References

    1. 1.CPT® Codes and Descriptions. AAPC. Retrieved Aug 11, 2026.Maintains the CPT code set's official descriptions and guidelines, licensed from the American Medical Association, used as the definitional source for CPT codes on this page.
    2. 2.HCPCS Quarterly Update. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's quarterly release of HCPCS Level II codes and short/long descriptors, the source for HCPCS code definitions and update history.
    3. 3.ICD-10-CM Full Code List. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's complete ICD-10-CM diagnosis code list, used to cross-reference diagnosis codes associated with this procedure or service.
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