ORPHA:48377
Subcorneal pustular dermatosis
Synonyms: Pustulosis subcornealis · Sneddon-Wilkinson disease · Subcorneal pustular dermatitis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare, acquired, chronic neutrophilic dermatosis characterized by sterile superficial pustules, typically involving the flexural sites of the trunk and proximal extremities.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Subcorneal pustular dermatosis | Pustulosis subcornealis · Sneddon-Wilkinson disease · Subcorneal pustular dermatitis |
| fr | Dermatose pustuleuse sous-cornée | Maladie de Sneddon-Wilkinson · Pustulosis subcornealis |
| de | Dermatose, pustulöse subkorneale | Sneddon-Wilkinson-Syndrom · Subkorneale Pustulosis |
| es | Dermatosis pustulosa subcórnea | Dermatitis pustulosa subcórnea · Enfermedad de Sneddon-Wilkinson · Pustulosis subcórnea |
| it | Dermatosi pustolosa subcorneale | Dermatite pustolosa subcorneale · Malattia di Sneddon-Wilkinson · Pustulosis subcornealis |
| nl | Subcorneale pustuleuze dermatose | Pustulosis subcornealis · Subcorneale pustuleuze dermatitis · Ziekte van Sneddon-Wilkinson |
| pt | Dermatose pustular sub-corneana | Doença de Sneddon-Wilkinson |
| pl | Podrogowa dermatoza krostkowa | Choroba Sneddona i Wilkinsona · Podrogowe krostkowe zapalenie skóry |
| cs | Subkorneální pustulární dermatóza | Pustulosis subcornealis · Sneddonova-Wilkinsonova nemoc · Subkorneální pustulární dermatitida · Dermatitis pustulosa subcornealis · Sneddon-Wilkinsonova nemoc |
| uk | Субкорнеальний пустульозний дерматоз | Пустульоз субкорнеальний · Хвороба Снеддона-Вілкінсона · Підшкірний пустульозний дерматит |
| zh | 角质层下脓疱皮肤病 | 角膜下脓疱性皮炎 |
| tr | Subkorneal püstüler dermatoz | Püstülozis subkornealis · Sneddon-Wilkinson hastalığı · Subkorneal püstüler dermatit |
Definition in:
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Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L13.1 | E | specific | validated |
| ICD-11 | EB2Y | NTBT | index | validated |
| MONDO | 0006614 | E | validated | |
| UMLS | C0600336 | E | validated | |
| GARD | 13606 | E | validated | |
| MedDRA | 10042342 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:90077Other acquired skin disease
rare skin diseases
No children.
4
Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 200 cases | Worldwide | Case(s) | Validated | ORPHANET | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | 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.
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HPO phenotypes
11 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0010783 | Erythema | |
| HP:0000953 | Hyperpigmentation of the skin | |
| HP:0200039 | Pustule | |
| Frequent (79-30%) | ||
| HP:0000989 | Pruritus | |
| Occasional (29-5%) | ||
| HP:0002960 | Autoimmunity | |
| HP:0000836 | Hyperthyroidism | |
| HP:0000821 | Hypothyroidism | |
| HP:0010702 | Increased circulating immunoglobulin concentration | |
| HP:0006775 | Multiple myeloma | |
| HP:0001370 | Rheumatoid arthritis | |
| HP:0002725 | Systemic lupus erythematosus | |
Run the differential on the 3 obligate / very frequent terms
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/48377
- This record as JSON: /data/d/48377.json