Familial partial lipodystrophy

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Familial partial lipodystrophy, Dunnigan type

ORPHA:2348Disease

Also called Dunnigan syndrome · FPLD2 · Familial partial lipodystrophy type 2

What it is

A rare, genetic lipodystrophy characterized by a loss of subcutaneous adipose tissue from the trunk, buttocks and limbs; fat accumulation in the neck, face, axillary and pelvic regions; muscular hypertrophy; and usually associated with metabolic complications such as insulin resistance, diabetes mellitus, dyslipidemia and liver steatosis.

Key facts

Age of onset
Adolescent, Adult, Childhood, Elderly
Inheritance
Autosomal dominant
Classified as
Disease

Recorded for the broader condition

Prevalence
1-9 / 1 000 000 (Europe)Familial partial lipodystrophy

Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.

Signs and symptoms

These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.

Gene

LMNADisease-causing germline mutation(s)

ICD-10 codes

E88.1filed under a broader ICD-10 category — shared with 24 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

GARD 3126MONDO 0007906OMIM 151660UMLS C1720860

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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