Panniculitis-induced localized lipodystrophy
- Type
- Disease
- Group
- Disorder
- Age of onset
- All ages
- Status
- active
Panniculitis-induced localized lipodystrophy is a rare, acquired, localized lipodystrophy disorder characterized by eruption of tender, occasionally painful, erythematous nodules and plaques which enlarge radially and resolve into lipoatrophic lesions, often located in the upper and lower limbs. Histologically, lesions are characterized by lipophagic, lobular panniculitis and absence of vasculitis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Panniculitis-induced localized lipodystrophy | |
| fr | Lipodystrophie localisée induite par une panniculite | |
| de | Lokale Lipodystrophie durch Pannikulitis | |
| es | Lipodistrofia localizada inducida por paniculitis | |
| it | Lipodistrofia localizzata indotta dalla panniculite | |
| nl | Panniculitis-geïnduceerde gelokaliseerde lipodystrofie | |
| pt | Paniculite e lipodistrofia localizada | |
| pl | Zapalenie tkanki podskórnej i lipodystrofia zlokalizowana | |
| cs | Panniculitida a lokalizovaná lipodystrofie | |
| uk | Локалізована ліподистрофія, спричинена панікулітом | |
| zh | 脂膜炎诱导局限性脂肪营养不良 | |
| tr | Panikülite bağlı 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 | index | validated |
| MONDO | 0019555 | E | validated | |
| MONDO | 19555 | E | validated | |
| UMLS | C5190785 | 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 | Unknown | — | 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
9 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:0010701 | Abnormal immunoglobulin level | |
| HP:0007485 | Absence of subcutaneous fat | |
| HP:0010783 | Erythema | |
| HP:0011123 | Inflammatory abnormality of the skin | |
| HP:0200036 | Skin nodule | |
| Occasional (29-5%) | ||
| HP:0003493 | Antinuclear antibody positivity | |
| HP:0200029 | Vasculitis in the skin | |
Run the differential on the 2 obligate / very frequent terms
Annotation source: 2613949[PMID]_3510652[PMID]_15554737[PMID]_18333902[PMID]_21865368[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/90159
- This record as JSON: /data/d/90159.json