Centrifugal lipodystrophy
Synonyms: Lipodystrophia centrifugalis abdominalis infantilis
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adolescent, Adult, Childhood, Elderly, Infancy
- Status
- active
Centrifugal lipodystrophy is a rare, acquired, localized lipodistrophy characterized by single or, occasionally, multiple, centrifugally progressive, asymptomatic to sometimes mildly tender, hypopigmented, lipoatrophic skin depressions with weakly erymatheous inflammatory borders, typically associated with regional ipsilateral lymph nodes swelling. Lesions typically occur on lower trunk (in particular groin and abdomen region), followed by upper trunk (axilla and neighboring regions) and, rarely, neck and head. It is usually not associated with systemic disease and is typically self-resolving.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Centrifugal lipodystrophy | Lipodystrophia centrifugalis abdominalis infantilis |
| fr | Lipodystrophie centrifuge | |
| de | Lipodystrophie, zentrifugale | = en |
| es | Lipodistrofia centrífuga | Lipodistrofia centrífuga abdominal infantil · Lipodistrophia centrifugalis abdominalis infantilis |
| it | Lipodistrofia centrifuga | Lipodistrofia centrifuga addominale infantile |
| nl | Centrifugale lipodystrofie | = en |
| pt | Lipodistrofia centrífuga | |
| pl | Lipodystrofia odśrodkowa | = en |
| cs | Centrifugální lipodystrofie | = en |
| uk | Відцентрова ліподистрофія | Відцентрова абдомінальна ліподистрофія немовлят |
| zh | 离心性脂肪代谢障碍 | 幼儿腹部离心性脂肪营养不良 |
| tr | Santrifüjel lipodistrofi | Lipodistrofi sentrifugalis abdominalis infantilis |
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 | index | validated |
| MONDO | 0019552 | E | validated | |
| UMLS | C0406605 | 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
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | 22211325[PMID]_ORPHANET |
| Cases/families | 168 cases | Worldwide | Case(s) | Validated | 22211325[PMID] |
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
10 annotations · validated 2017-04-19| 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:0000765 | Abnormal thorax morphology | |
| HP:0010783 | Erythema | |
| HP:0011123 | Inflammatory abnormality of the skin | |
| HP:0002840 | Lymphadenitis | |
| HP:0040189 | Scaling skin | |
| Very rare (<4-1%) | ||
| HP:0001596 | Alopecia | |
| HP:0005320 | Lack of facial subcutaneous fat | |
Run the differential on the 2 obligate / very frequent terms
Annotation source: 7073970[PMID]_6691914[PMID]_8136542[PMID]_12080947[PMID]_17894715[PMID]_19712277[PMID]_20811202[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/90156
- This record as JSON: /data/d/90156.json