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    OpenPayerBilling CodesOther congenital malformationsICD-10-CM q81-epidermolysis-bullosa
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    ICD-10-CM Code For Epidermolysis Bullosa: Group Overview

    The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group Q81 includes congenital blistering disorders characterized by skin fragility, blister formation, and varying involvement of mucous membranes and other tissues, encompassing multiple subtypes of epidermolysis bullosa. These ICD-10-CM diagnosis codes are used to document clinical scenarios including disease severity, presence of complications such as infection or scarring, and involvement of different anatomical sites. Accurate coding within the ICD-10-CM Q81 group supports proper claim adjudication, complete clinical documentation, and appropriate reimbursement.

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    ICD-10-CM Q81: Epidermolysis Bullosa Overview

    The ICD-10-CM Q81 group represents congenital blistering disorders of the skin known collectively as epidermolysis bullosa. These conditions affect the integumentary system, causing skin fragility, blistering, and in some subtypes mucosal involvement. Accurate coding is important to reflect disease severity and complications, which supports appropriate reimbursement and continuity of care.

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    When to Use

    Use a code from this group when documenting a diagnosis of epidermolysis bullosa confirmed by clinical findings, family history, or specialized testing that identifies a specific subtype. Codes from this group are applied to encounters addressing acute blistering, chronic management, wound care, or complications related to the disorder.

    ICD-10-CM Codes

    5 rows

    ICD-10-CM CodeDescription
    Q81.0Epidermolysis bullosa simplex
    Q81.1Epidermolysis bullosa letalis
    Q81.2Epidermolysis bullosa dystrophica
    Q81.8Other epidermolysis bullosa
    Q81.9Epidermolysis bullosa, unspecified

    DRG Codes

    2 rows

    DRG CodeDescription
    595Major Skin Disorders with MCC
    596Major Skin Disorders without MCC

    Typical Clinical Scenarios

    • A neonate is born with widespread blistering and areas of denuded skin noted at delivery and during initial newborn exam; the infant requires neonatal intensive care for fluid management, wound care, and prevention of infection. A congenital mechanobullous disorder is suspected based on family history and the distribution of lesions, so a specific epidermolysis bullosa subtype diagnosis is assigned for inpatient care and genetic workup. Typical codes: Q81.0, Q81.9
    • An adolescent with a lifelong history of fragile skin presents to dermatology clinic for routine management of chronic non-healing erosions, mucosal involvement, and scarring that limit mobility; care focuses on specialized wound care, infection prevention, and monitoring for complications. Because the patient has a persistent inherited epidermolysis bullosa variant documented in prior records, the encounter is coded to reflect the long-standing congenital blistering disorder. Typical codes: Q81.1, Q81.2
    • An adult with known congenital epidermolysis bullosa is admitted from clinic with a superimposed skin infection and painful cellulitis in areas of recurrent blistering after minor trauma; the acute infectious complication requires systemic antibiotics and inpatient dermatology and infectious disease consultation. Coding reflects both the underlying hereditary mechanobullous condition and the acute infectious complication, assigning the appropriate code for the underlying epidermolysis bullosa as part of the diagnosis list. Typical codes: Q81.8, Q81.2

    Related Codes

    Q80-CONGENITAL-ICHTHYOSIS

    ICD-10-CM Code For Congenital Ichthyosis: 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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