Necrobiosis lipoidica
Synonyms: Oppenheim-Urbach disease
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood, Elderly
- Status
- active
A rare skin disease characterized by enlarging, annular plaques with red-brown edges and atrophic, yellow-brown, telangiectatic centers. The lesions are commonly asymptomatic, but affected skin areas may be fragile, and painful ulcerations develop in many cases. In rare cases, development of squamous cell carcinoma within longstanding lesions has been reported. The lower legs, especially the shins, are the most frequently involved site. The condition is often associated with diabetes mellitus.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Necrobiosis lipoidica | Oppenheim-Urbach disease |
| fr | Nécrobiose lipoïdique | Dermatite atrophiante lipoïdique d'Oppenheim-Urbach · Maladie d'Oppenheim-Urbach |
| de | Necrobiosis lipoidica | Oppenheim-Urbach-Syndrom |
| es | Necrobiosis lipoídica | Enfermedad de Oppenheim-Urbach |
| it | Necrobiosi lipoidica | |
| nl | Necrobiosis lipoidica | Ziekte van Oppenheim-Urbach |
| pt | Necrobiose lipóidica | |
| pl | Obumieranie tłuszczowate | = en |
| cs | Necrobiosis lipoidica | Oppenheimova-Urbachova nemoc · Oppenheim-Urbachova nemoc |
| uk | Ліпоїдний некробіоз | Хвороба Оппенгейма-Урбаха |
| zh | 类脂性渐进性坏死 | Oppenheim-Urbach疾病 |
| tr | Nekrobiosis lipoidika | Oppenheim-Urbach hastalığı |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L92.1 | E | specific | validated |
| ICD-11 | EE80.1 | E | specific | validated |
| MONDO | 0006583 | E | validated | |
| MeSH | D009335 | E | validated | |
| UMLS | C0027538 | E | validated | |
| GARD | 13040 | 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:79381Other dermis disorder
rare skin 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_29184811[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
21 annotations · validated 2021-06-03| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0025528 | Annular cutaneous lesion | |
| HP:0011123 | Inflammatory abnormality of the skin | |
| Frequent (79-30%) | ||
| HP:0011990 | Abnormality of neutrophil physiology | |
| HP:0002814 | Abnormality of the lower limb | |
| HP:0001075 | Atrophic scars | |
| HP:0000819 | Diabetes mellitus | |
| HP:0010783 | Erythema | |
| HP:0001030 | Fragile skin | |
| HP:0032252 | Granuloma | |
| HP:0032217 | Indurated nodule | |
| HP:0200034 | Papule | |
| HP:0200036 | Skin nodule | |
| HP:0200035 | Skin plaque | |
| HP:0200042 | Skin ulcer | |
| HP:0100585 | Telangiectasia of the skin | |
| Occasional (29-5%) | ||
| HP:0000271 | Abnormality of the face | |
| HP:0002973 | Abnormality of the forearm | |
| HP:0001155 | Abnormality of the hand | |
| HP:0001965 | Abnormality of the scalp | |
| Very rare (<4-1%) | ||
| HP:0000820 | Abnormality of the thyroid gland | |
| HP:0002860 | Squamous cell carcinoma | |
Run the differential on the 2 obligate / very frequent terms
Annotation source: 32023016[PMID]_27686584[PMID]_26143418[PMID]_31719288[PMID]_29352388[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/542592
- This record as JSON: /data/d/542592.json