Focal acral hyperkeratosis
Synonyms: PPKP3 without elastoidosis · PPPK3 without elastoidosis · Punctate palmoplantar hyperkeratosis type 3 without elastoidosis · Punctate palmoplantar keratoderma type 3 without elastoidosis
- Type
- Disease
- Group
- Disorder
- Status
- active
A rare epidermal disease characterized by multiple, usually asymptomatic, yellowish to flesh colored hyperkeratotic papules and plaques on the palms and soles, with a preference for the palmar and plantar margins. Histological examination shows pronounced orthohyperkeratosis overlying a crateriform depression in the epidermis, with hypergranulosis and mild acanthosis, while elastorrhexis is absent. The lesions appear in the second or third decade of life and gradually increase in number over several years. The condition may be sporadic or familial.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Focal acral hyperkeratosis | PPKP3 without elastoidosis · PPPK3 without elastoidosis · Punctate palmoplantar hyperkeratosis type 3 without elastoidosis · Punctate palmoplantar keratoderma type 3 without elastoidosis |
| fr | Hyperkératose acrale focale | Hyperkératose palmoplantaire ponctuée type 3 sans élastoïdose · KPPP3 sans élastoïdose · Kératodermie palmoplantaire ponctuée type 3 sans élastoïdose |
| de | Hyperkeratose, akrale fokale | PPKP3 ohne Elastoidose · Palmoplantare Hyperkeratose, punktierte, Typ 3, ohne Elastoidose · Palmoplantarkeratose, punktierte, Typ 3, ohne Elastoidose |
| es | Hiperqueratosis acral focal | Elastoidosis sin PPKP3 · Elastoidosis sin PPPK3 · Hiperqueratosis palmoplantar punctata tipo 3 sin elastoidosis · Queratodermia palmoplantar punctata tipo 3 sin elastoidosis |
| it | Ipercheratosi acrale focale | Cheratoderma palmoplantare puntato tipo 3, senza elastoidosi · Ipercheratosi palmoplantare puntata tipo 3, senza elastoidosi · PPKP3 senza elastoidosi · PPPK3 senza elastoidosi |
| nl | Focale acrale hyperkeratose | PPKP3 zonder elastoïdose · PPPK3 zonder elastoïdose · Puntvormige palmoplantaire hyperkeratose type 3 zonder elastoïdose · Keratoderma palmoplantaris punctata type 3 zonder elastoïdose |
| pt | Hiperqueratose focal acral | |
| pl | Hiperkeratoza ogniskowa kończynowa | Hiperkertoza dłoniowo-podeszwowa punktowa typu 3 bez elastoidozy · PPKP3 bez elastoidozy · PPPK3 bez elastoidozy · Rogowiec dłoniowo-podeszwowy punktowy typu 3 bez elastoidozy |
| cs | Fokální akrální hyperkeratóza | PPKP3 bez elastoidózy · PPPK3 bez elastoidózy · Keratosis palmoplantaris punctata, typ 3, bez elastoidózy |
| uk | Фокальний акральний гіперкератоз | PPKP3 без еластоїдозу · PPPK3 без еластоїдозу · Пунктирний долонно-підошовний гіперкератоз типу 3 без еластоїдозу · Пунктирна долонно-підошовна кератодермія типу 3 без еластоїдозу |
| zh | 局灶性肢端角化过度 | 点状掌跖角化病3型不伴类弹性纤维病 |
| tr | Fokal akral hiperkeratoz | Elastoidoz ile seyretmeyen PPKP3 · Elastoidoz ile seyretmeyen PPPK3 · Elastoidoz ile seyretmeyen punktata palmoplantar keratoderma tip 3 · Elastoidoz ile seyretmeyen punktata palmoplantar hiperkeratoz tip 3 |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q82.8 | NTBT | attributed | validated |
| ICD-11 | EC20.32 | NTBT | attributed | validated |
| MONDO | 0017677 | E | validated | |
| UMLS | C1302839 | 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:307995Marginal papular palmoplantar keratoderma
rare genetic diseases; rare skin diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/308013
- This record as JSON: /data/d/308013.json