Zinc-responsive necrolytic acral erythema
Synonyms: Necrolytic acral erythema · NAE
- Type
- Particular clinical situation in a disease or syndrome
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare skin disease characterized initially by erythematous patches with superficial necrosis, at later stages by hyperkeratotic plaques with a rim of dusky erythema, of exclusively acral distribution with a predilection for the lower extremities, and universal association with hepatitis C virus infection. Patients typically experience pruritus or pain. Serum zinc levels may or may not be decreased, although oral zinc supplementation often improves the condition.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Zinc-responsive necrolytic acral erythema | Necrolytic acral erythema · NAE |
| fr | Erythème nécrolytique acral | ENA |
| de | Zink-responsive nekrolytisches akrales Erythem | NAE · Nekrolytisches akrales Erythem |
| es | Eritema necrolítico acral con respuesta al cinc | Eritema necrolítico acral · Eritema necrolítico acral con respuesta al zinc · NAE |
| it | Eritema acrale necrolitico sensibile allo zinco | Eritema acrale necrolitico · NAE |
| nl | Zinkresponsief necrolytisch acraal erytheem | NAE · Necrolytisch acraal erytheem |
| pt | Eritema necrolítico acral sensível ao zinco | |
| pl | Martwiczy rumień kończynowy wrażliwy na cynk | Martwiczy rumień kończynowy · NAE |
| cs | Nekrolytický akrální erytém citlivý na zinek | NAE · Nekrolytický akrální erytém |
| uk | Цинк-чутлива некролітична акральна еритема | NAE · Некротична акральна еритема |
| zh | 锌反应性坏死性肢端红斑 | 坏死性肢端红斑 |
| tr | Çinko duyarlı nekrolitik akral eritem | NAE · Nekrolitik akral eritem |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L53.8 | NTBT | attributed | validated |
| ICD-11 | EA20 | E | specific | validated |
| MONDO | 0018586 | E | validated | |
| UMLS | C4546437 | E | validated | |
| MedDRA | 10084061 | 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:79359Other epidermal 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 | Not yet 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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/439196
- This record as JSON: /data/d/439196.json