Idiopathic localized lipodystrophy
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adolescent, Adult, Childhood, Infancy
- Status
- active
Idiopathic localized lipodystrophy is a rare, acquired, localized lipodystrophy characterized by asymptomatic, well-demarcated, depressed, lipoatrophic lesions of variable size, with normal overlying skin without antecedent inflammation or a known identifiable cause (autoimmune disease, drug injection, injury, etc).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Idiopathic localized lipodystrophy | |
| fr | Lipodystrophie localisée idiopathique | |
| de | Lipodystrophie, lokale, idiopathische | |
| es | Lipodistrofia idiopática localizada | |
| it | Lipodistrofia localizzata idiopatica | |
| nl | Idiopathische gelokaliseerde lipodystrofie | |
| pt | Lipodistrofia localizada idiopática | |
| pl | Idiopatyczna zlokalizowana lipodystrofia | |
| cs | Idiopatická lokalizovaná lipodystrofie | |
| uk | Ідіопатична локалізована ліподистрофія | |
| zh | 特发性局限性脂质营养不良 | |
| tr | İdiyopatik lokalize lipodistrofi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | E88.1 | NTBT | attributed | validated |
| ICD-11 | EF01.1 | NTBT | attributed | validated |
| MONDO | 0019554 | E | validated | |
| UMLS | C0473566 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:79088Localized lipodystrophy
rare skin diseases; rare endocrine diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | — | Worldwide | Class only | Validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
11 annotations · validated 2016-09-28| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0100578 | Lipoatrophy | |
| Very frequent (99-80%) | ||
| HP:0003758 | Reduced subcutaneous adipose tissue | |
| Frequent (79-30%) | ||
| HP:0007485 | Absence of subcutaneous fat | |
| Occasional (29-5%) | ||
| HP:0010783 | Erythema | |
| HP:0000953 | Hyperpigmentation of the skin | |
| HP:0001010 | Hypopigmentation of the skin | |
| HP:0011123 | Inflammatory abnormality of the skin | |
| HP:0000989 | Pruritus | |
| Very rare (<4-1%) | ||
| HP:0012344 | Morphea | |
| HP:0040189 | Scaling skin | |
| HP:0100324 | Scleroderma | |
Run the differential on the 2 obligate / very frequent terms
Annotation source: 8859277[PMID]_24346897[PMID]_18718199[PMID]_18280348[PMID]_17989899[PMID]_18718199[PMID]_8252774[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/90158
- This record as JSON: /data/d/90158.json