2026 ICD-10-CM Diagnosis Code E88.12Generalized lipodystrophy

ICD-10-CM Codes›E00–E89›E70-E88›E88

ICD-10-CM E88.12
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E88.12 is a billable ICD-10-CM diagnosis code for generalized lipodystrophy. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026).

Code Identity

ICD-10-CM Code
E88.12
Billable Status
Yes — Valid for Submission
Code Describes
Generalized lipodystrophy
Short Description
Generalized lipodystrophy
Same as the full description in the CMS dataset.
Parent Code
E88.1
Lipodystrophy, not elsewhere classified

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE88Other and unspecified metabolic disorders
This CodeE88.12Generalized lipodystrophy

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Acquired generalized lipodystrophy
  • Congenital abnormal shape of clavicle
  • Congenital anomaly of subcutaneous tissue
  • Congenital deformity of clavicle
  • Congenital deformity of shoulder
  • Congenital generalized lipodystrophy
  • Generalized congenital lipodystrophy with myopathy
  • Keppen Lubinsky syndrome
  • Mandibuloacral dysostosis
  • Mandibuloacral dysplasia with type B lipodystrophy

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Acquired generalized lipodystrophy (AGL)
  • Berardinelli-Siep syndrome
  • Congenital generalized lipodystrophy (CGL)
  • Lawrence syndrome

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • AGL(acquired generalized lipodystrophy)
    • CGL(congenital generalized lipodystrophy)
    • Lipodystrophy
      • generalized (acquired) (congenital)
    • Syndrome
      • Berardinelli-Siep
    • Syndrome
      • Lawrence

Clinical InformationClinical

  • Acquired Generalized Lipodystrophy

    generalized lipodystrophy, the cause of which is not present at birth.

  • Berardinelli-Seip Congenital Lipodystrophy|Berardinelli Lipodystrophy Syndrome|Berardinelli Seip Syndrome|Congenital Generalized Lipodystrophy|Congenital Generalized Lipodystrophy|Lawrence-Seip Syndrome|Seip-Bernardinelli Syndrome|Total Lipodystrophy

    a genetic disorder caused by mutations in the agpat2 and bscl2 genes. it is characterized by lipoatrophy in the trunk, face, and extremities, hypertriglyceridemia, muscle hypertrophy, cardiomyopathy and hepatomegaly. patients develop insulin resistance, leading to diabetes mellitus.

  • CAVIN1 wt Allele|CAVIN|CGL4|Caveolae Associated Protein 1 wt Allele|Cavin-1 Gene|Congenital Generalized Lipodystrophy 4 Gene|FKSG13|PTRF|RNA Polymerase I and Transcript Release Factor Gene|TTF-I Interacting Peptide 12 Gene

    human cavin1 wild-type allele is located in the vicinity of 17q21.2 and is approximately 21 kb in length. this allele, which encodes caveolae-associated protein 1, plays a role in the regulation of rrna transcription and the formation of caveolae. mutations in the gene are associated with congenital generalized (berardinelli-seip) lipodystrophy 4.

  • Generalized Lipodystrophy

    almost complete absence of subcutaneous and/or visceral adipose tissue.

Code History & ChangesHistory

New Code E88.12 was added to the ICD-10-CM code set for FY 2026, effective October 1, 2025.

Replacement E88.12 replaces the following previously assigned code(s):

  • E88.1 - Lipodystrophy, not elsewhere classified
FY 2026AddedAdded to the ICD-10-CM code setEffective October 1, 2025.
FY 2026CurrentRevised in the current code setEffective October 1, 2025 through September 30, 2026.

Questions About E88.12Overview

Is E88.12 (Lipodystrophy, not elsewhere classified) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report generalized lipodystrophy on HIPAA-covered claims from October 1, 2025 through September 30, 2026.