Pustulosis palmaris et plantaris
Synonyms: Palmoplantar pustulosis · LPP · Localized pustular psoriasis · PPP
- Type
- Disease
- Group
- Disorder
- Inheritance
- Autosomal dominant, Autosomal recessive
- Status
- active
A rare skin disease characterized by chronic eruption of sterile pustules on an erythematous and desquamative background. The lesions are usually painful and affect the palms and soles, sometimes also the lateral aspects of hands and feet. Nail lesions (such as nail pitting, onycholysis, subungual pustules, and nail dystrophy) are also observed. The condition takes a chronic and relapsing course. Typical associations are psoriatic arthritis, thyroid gland dysfunction, and smoking.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Pustulosis palmaris et plantaris | Palmoplantar pustulosis · LPP · Localized pustular psoriasis · PPP |
| fr | Pustulose palmoplantaire | PPL · PPP · Psoriasis pustuleux localisé |
| de | Pustulosis Palmaris et Plantaris | |
| es | Pustulosis palmar y plantar | PPP · Psoriasis pustulosa localizada · Pustulosis palmoplantar |
| it | Pustolosi palmo-plantare | LPP · PPP · Psoriasi pustolosa localizzata · Pustolosi palmoplantare |
| nl | Pustulosis palmaris et plantaris | Gelokaliseerde pustuleuze psoriasis · LPP · PPP · Palmoplantaire pustulose |
| pt | Pustulosis palmaris et plantaris | |
| pl | Łuszczyca krostkowa dłoni i podeszw | Krostkowica dłoni i podeszw · LPP · Ograniczona łuszczyca krostkowa · PPP |
| cs | Pustulosis palmaris et plantaris | LPP · Lokalizovaná pustulózní psoriáza · PPP · Palmoplantární pustulóza |
| uk | Пустульоз долонь і підошов | Локалізований пустульозний псоріаз · LPP · Пальмоплантарний пустульоз · РРР |
| zh | 掌跖脓疱病 | 局限性脓疱型银屑病 |
| tr | Püstüloz palmaris et plantaris | LPP · PPP · Palmoplantar püstüloz · Lokalize püstüler sedef hastalığı |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L40.3 | E | specific | validated |
| ICD-11 | EA90.42 | E | specific | validated |
| MONDO | 0015597 | E | validated | |
| UMLS | C0030246 | E | validated | |
| GARD | 12820 | E | validated | |
| MedDRA | 10050185 | 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:79359Other epidermal disorder
rare skin diseases - ORPHA:79360Other genetic epidermal disease
rare genetic diseases
No children.
Genes
2 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| AP1S3 | Disease-causing germline mutation(s) in | Assessed | HGNC:18971 | ENSG00000152056 | 615781 | 2q36.1 | adaptor related protein complex 1 subunit sigma 3 | 24791904[PMID] |
| IL36RN | Disease-causing germline mutation(s) in | Assessed | HGNC:15561 | ENSG00000136695 | 605507 | 2q14.1 | interleukin 36 receptor antagonist | 23303454[PMID] |
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/163927
- This record as JSON: /data/d/163927.json