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Q81.1 ICD-10-CM Code: Epidermolysis bullosa letalis

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Billing Status: YES. This is a valid, specific, and billable ICD-10-CM reference.

Coding at a Glance

Tabular directives
1 inclusion term

Billable · FY2027A valid, specific ICD-10-CM code, reportable for dates of service in FY2027.

What you need to know

Source: CMS/NCHS Official ICD-10-CM tabular notes, quoted. From the CMS/NCHS tabular list for the release in force. A note the category or block publishes applies to this code too; the Instructions section marks which is which.

IncludesWhat this code covers
  • Herlitz' syndrome

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Inpatient Payment Groups (MS-DRG)

Potential MS-DRG participation — not a DRG assignment.

MS-DRGs this diagnosis helps define, as principal or secondary, per the CMS ICD-10-CM/PCS MS-DRG Definitions Manual v44, Appendix B.

  • MS-DRG 595 — MAJOR SKIN DISORDERS WITH MCC (MDC 09)
  • MS-DRG 596 — MAJOR SKIN DISORDERS WITHOUT MCC (MDC 09)

A diagnosis appearing in a group's logic does not by itself determine the DRG assigned to a stay; the grouper uses the full claim.

Coding instructions

Official Tabular Instructional Notes: the inclusion, exclusion and sequencing notes published for Q81.1 in the official ICD-10-CM tabular list, quoted as published.

Source: CMS/CDC — ICD-10-CM Tabular ListRelease: FY2027Effective: October 1, 2026

Trace:FY2027 changesChange historyRelease, file and checksum

Inclusion Terms

Alternative terms the tabular list files under this code.

  • Herlitz' syndrome

Don’t confuse Q81.1 with…

MedCoder analysis — pairs from this code’s official notes, distinctions written by MedCoder

The codes most likely to be picked instead of Q81.1 — named in its own official notes, or sharing its laterality or encounter family — with what separates them and which documentation moves the case. Each links to the full comparison.

  • Q81.9— Specificity relationship (Review)

    Overlapping diagnoses in the same category.

    Overlapping codes in the same category — the documentation decides which is the more specific fit.

    What moves you there: A record that documents no more than “Epidermolysis bullosa, unspecified” takes Q81.9; specificity in the record moves it back here.

    Full comparison: Q81.1 vs Q81.9 →

Relationships are read from this code's own official notes, its laterality siblings and its encounter family; the wording of each distinction is MedCoder editorial, and the documentation named in each “what moves you there” line is the official note phrase or sibling title itself. Source: CMS ICD-10-CM tabular instructional notes; ICD-10-CM Official Guidelines, Section I.A.12.a.

Coding context

Guidelines, coding notes, decision aids, relationships (with MS-DRG and CCSR classification), hierarchy, HCC, coverage and the context map: what a coder reaches for after the core. Each section names whether it is official source data, a MedCoder-derived relationship or MedCoder editorial.

Code Overview

Epidermolysis bullosa letalis is a billable ICD-10-CM diagnosis code (Q81.1).

MedCoder summary Summary composed by MedCoder from this code's official ICD-10-CM record. The tabular instructional notes themselves appear verbatim below.

Verify Before Coding

  • No Medicare Code Editor or MS-DRG Definitions Manual restrictions apply to this code.

From the code registry, the Medicare Code Editor, and the MS-DRG Definitions Manual. Check it against a full claim in Claim Check.

Relationships & Classification

MedCoder-derived relationships — computed from published CMS and AHRQ datasets

Other codes that name Q81.1 or its code family, from the CMS ICD-10-CM tabular instructional notes. Tabular-note edges are stored at the code family level that carries each note.

Referenced by 2 Excludes1 notes across 2 chapters: L12.3 — Acquired epidermolysis bullosa (via Q81.-), P83 — Other conditions of integument specific to newborn (via Q81.-).

These codes’ tabular lists mark this diagnosis as mutually exclusive — not reported together unless the conditions are documented as unrelated.

MS-DRG Grouper Relationships (FY2027)

Potential MS-DRG participation — not a DRG assignment.

FY2027 MS-DRG: not on the CMS CC/MCC list — as a secondary diagnosis this code does not change MS-DRG severity for that release.

Named in the grouper logic of 2 MS-DRGs: DRG 595 (MDC 09), DRG 596 (MDC 09).

From the CMS MS-DRG Definitions Manual (Appendices B and C). Actual DRG assignment depends on the complete claim.

Clinical classification (AHRQ CCSR):MAL010 — Other specified and unspecified congenital anomalies (default).

Clinical Classifications Software Refined (CCSR) for ICD-10-CM Diagnoses. Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality.

Related Codes

Same clinical category (CCSR)

AHRQ's Clinical Classifications Software groups these diagnoses under the same clinical category (Other specified and unspecified congenital anomalies).

P09.2 — Abnormal findings on neonatal screening for congenital endocrine disease, P09.3 — Abnormal findings on neonatal screening for congenital hematologic disorders, Q80.0 — Ichthyosis vulgaris, Q80.1 — X-linked ichthyosis, Q80.2 — Lamellar ichthyosis, Q80.3 — Congenital bullous ichthyosiform erythroderma, Q80.4 — Harlequin fetus, Q80.8 — Other congenital ichthyosis, Q80.9 — Congenital ichthyosis, unspecified, Q81.0 — Epidermolysis bullosa simplex, Q81.2 — Epidermolysis bullosa dystrophica, Q81.8 — Other epidermolysis bullosa, Q81.9 — Epidermolysis bullosa, unspecified, Q82.0 — Hereditary lymphedema, Q82.1 — Xeroderma pigmentosum, Q82.2 — Congenital cutaneous mastocytosis, Q82.3 — Incontinentia pigmenti, Q82.4 — Ectodermal dysplasia (anhidrotic), Q82.5 — Congenital non-neoplastic nevus, Q82.6 — Congenital sacral dimple, +59 more

Same Index main term, other category

The ICD-10-CM Index to Diseases and Injuries files this code under the main term “Epidermolysis”; these codes share that main term but sit in a different category of the Tabular List.

L12.30 — Acquired epidermolysis bullosa, unspecified (bullosa, acquired), L12.31 — Epidermolysis bullosa due to drug (bullosa, acquired, drug-induced), L12.35 — Other acquired epidermolysis bullosa (bullosa, acquired, specified cause NEC), L51.2 — Toxic epidermal necrolysis [Lyell] (necroticans combustiformis)

Contextual Map

Every relationship of Q81.1 in one view: hierarchy, official tabular instructions in both directions, clinical classification, risk adjustment, MS-DRG participation, index terms and change history — each edge carrying the CMS source it derives from.

Run Q81.1 with these 2 related codes in Claim Check

Hierarchy

Referenced by Excludes1 notes

Clinical classification (CCSR)

MS-DRG Grouper

MDC crossing

  • MDC 09 — Diseases and Disorders of the Skin, Subcutaneous Tissue and Breast[MDC crossing]: “Diseases and Disorders of the Skin, Subcutaneous Tissue and Breast — the grouper's crossing between diagnoses and procedures: a principal diagnosis sets the MDC, and same-MDC procedures move the stay to its surgical DRGs. 7,378 same-MDC procedures group here; browse them on the MDC page.”— CMS MS-DRG Definitions Manual · FY2027

Index entries

  • Epidermolysis, bullosa (congenital), letalis[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027
  • Herlitz' syndrome[Index term]— CMS ICD-10-CM Index to Diseases and Injuries · icd10cm-fy2027

Nearest codes

Change history

  • FY2016 — In the code set at ICD-10-CM adoption [Change history]— CMS release files (code change ledger) · icd10cm-fy2016

Reference

Index terms and tables, published questions and FAQ, every source behind this page with its release and checksum, the date-of-service check and the complete change history.

Indexed Clinical Terms (2)

Official source data — entries quoted as published, in the Index’s own lookup phrasing

Clinical term phrases from the official ICD-10-CM Index to Diseases and Injuries that map to this code. These are alphabetic-index entries shown as the Index writes them — lookup phrasing, not necessarily the wording of a final diagnosis.

Sources for this page

Codes, titles, notes, index terms and mappings on this page are transcribed from the datasets below. Relationships MedCoder computed and text MedCoder wrote are labelled where they appear.

Code, title, tabular notes and index terms Official source data
CMS/CDC ICD-10-CM FY2027 tabular list, index and tables, effective October 1, 2026 Release, file and checksum · Publisher’s page
Claim edits Official source data
CMS Definitions of Medicare Code Edits — v44.0 (October 2026) Release, file and checksum · Publisher’s page
Inpatient payment groups Official source data
CMS MS-DRG Definitions Manual (incl. Appendix B diagnosis index, Appendix C CC/MCC list, Appendix E procedure index) and IPPS Final Rule tables — v44 Release, file and checksum · Publisher’s page
Change history and date-of-service validity Official source data
CMS ICD-10-CM release addenda, ingested release by release into the change ledger Release, file and checksum · Publisher’s page
Comparisons, relationships and the contextual map MedCoder-derived relationship
Computed by MedCoder from the tabular notes and tables above; every derived relationship is marked as derived where it appears
Summary and FAQ answers MedCoder editorial explanation
Written by MedCoder to explain the sources above: drafted with AI assistance, checked by a person against the release files, and labelled as MedCoder text where it appears. Not official text.

Current data releases:ICD-10-CM FY2027 · ICD-10-PCS FY2027 · HCPCS October 2026 · MS-DRG v44 · Medicare Code Editor v44.0 · NCCI PTP Q4 2026 · MUE Q4 2026 · NCD code lists 2026-01 · LCD export September 28, 2026 · All releases and sources

Labels on this page: Official source data · MedCoder-derived relationship · MedCoder editorial explanation. How to read the labels · All data sources and release dates · CMS coding rules

Cite this page

Reference this page in a research guide, syllabus or article. The release is included because code content changes each year.

MedCoder.ai. "Q81.1 — Epidermolysis bullosa letalis." ICD-10-CM FY2027. https://medcoder.ai/icd10/code/q81.1-epidermolysis-bullosa-letalis

Change history

  • FY2016 — October 1, 2015
    In the code set at ICD-10-CM adoption
    Epidermolysis bullosa letalis

No changes since FY2016 — additions, deletions, description changes and billable-status changes are tracked through FY2027, and none are recorded for this code. Note changes are tracked from FY2027 only.

Nearest Codes in This Family

Official ICD-10-CM classifications closest to Q81.1 in its code family, with their registry titles.

View all codes in the Q81 family