Acquired generalized lipodystrophy
Synonyms: Acquired lipoatrophic diabetes · Lawrence syndrome · Lawrence-Seip syndrome
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare lipodystrophic syndrome characterized by loss of adipose tissue, and is a syndrome of insulin resistance that leads to increased cardiovascular risk. Acquired generalized lipodystrophy is related to a selective loss of subcutaneous adipose tissue occurring exclusively at the extremities (face, legs, arms, palms and sometimes soles).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Acquired generalized lipodystrophy | Acquired lipoatrophic diabetes · Lawrence syndrome · Lawrence-Seip syndrome |
| fr | Lipodystrophie généralisée acquise | Diabète lipoatrophique acquis · Syndrome de Lawrence · Syndrome de Lawrence-Seip |
| de | Lipodystrophie, erworbene generalisierte | Diabetes, erworbener lipoatrophischer · Lawrence-Syndrom |
| es | Lipodistrofia generalizada adquirida | Diabetes lipoatrófica adquirida · Síndrome de Lawrence |
| it | Lipodistrofia generalizzata acquisita | Diabete lipoatrofico acquisito · Sindrome di Lawrence · Sindrome di Lawrence-Seip |
| nl | Verworven gegeneraliseerde lipodystrofie | Syndroom van Lawrence · Syndroom van Lawrence-Seip · Verworven lipoatrofische diabetes |
| pt | Lipodistrofia generalizada adquirida | Diabetes lipoatrófica adquirida · Síndrome de Lawrence |
| pl | Nabyta uogólniona lipodystrofia | Nabyta cukrzyca lipoatroficzna · Zespół Lawrence'a · Zespół Lawrence'a i Seipa |
| cs | Získaná generalizovaná lipodystrofie | Získaný lipoatrofický diabetes · Lawrencův syndrom · Lawrencův-Seipův syndrom · Lawrence-Seipův syndrom |
| uk | Набута генералізована ліподистрофія | Набутий ліпоатрофічний діабет · Синдром Лоуренса · Синдром Лоуренса-Сейпа |
| zh | 获得性全身性脂质营养不良 | 获得性脂肪萎缩性糖尿病 |
| tr | Edinsel jeneralize lipodistrofi | Lawrence sendromu · Lawrence-Seip sendromu · Edinsel lipoatrofik diyabet |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | E88.1 | NTBT | attributed | validated |
| ICD-11 | 5A44 | NTBT | index | validated |
| MONDO | 0019193 | E | validated | |
| UMLS | C0271693 | E | validated | |
| GARD | 12603 | E | validated | |
| MedDRA | 10087376 | 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 (2):
- ORPHA:98307Acquired lipodystrophy
rare skin diseases; rare endocrine diseases - ORPHA:181368Rare insulin-resistance syndrome
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 | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
| Point prevalence | 1-9 / 100 000 | 1 / 100 000 | Europe | Value and class | Validated | European Medicines Agency 2012[OTHER] |
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
28 annotations · validated 2016-09-05| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0009064 | Generalized lipodystrophy | |
| Very frequent (99-80%) | ||
| HP:0000842 | Hyperinsulinemia | |
| HP:0000855 | Insulin resistance | |
| Frequent (79-30%) | ||
| HP:0003119 | Abnormal circulating lipid concentration | |
| HP:0011025 | Abnormality of cardiovascular system physiology | |
| HP:0002960 | Autoimmunity | |
| HP:0003707 | Calf muscle pseudohypertrophy | |
| HP:0001638 | Cardiomyopathy | |
| HP:0001397 | Hepatic steatosis | |
| HP:0000831 | Insulin-resistant diabetes mellitus | |
| HP:0005328 | Progeroid facial appearance | |
| Occasional (29-5%) | ||
| HP:0005339 | Abnormality of complement system | |
| HP:0000956 | Acanthosis nigricans | |
| HP:0005616 | Accelerated skeletal maturation | |
| HP:0001735 | Acute pancreatitis | |
| HP:0001394 | Cirrhosis | |
| HP:0002230 | Generalized hirsutism | |
| HP:0007440 | Generalized hyperpigmentation | |
| HP:0002240 | Hepatomegaly | |
| HP:0000822 | Hypertension | |
| HP:0002155 | Hypertriglyceridemia | |
| HP:0003198 | Myopathy | |
| HP:0012490 | Panniculitis | |
| HP:0000147 | Polycystic ovaries | |
| HP:0000093 | Proteinuria | |
| Very rare (<4-1%) | ||
| HP:0009592 | Astrocytoma | |
| HP:0002665 | Lymphoma | |
| HP:0012064 | Unicameral bone cyst | |
Run the differential on the 3 obligate / very frequent terms
Annotation source: 11078466[PMID]_22748602[PMID]_25864863[PMID]_20551664[PMID]_19924491[PMID]_20616664[PMID]_19924491[PMID]_17097389[PMID]_26252356[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/79086
- This record as JSON: /data/d/79086.json