ORPHA:222
Erosive pustular dermatosis of the scalp
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood, Elderly
- Status
- active
Erosive pustular dermatosis of the scalp is a rare chronic inflammation of the scalp usually occurring in elderly women (>70 years old) and characterized by the development of painful pustules, shallow erosions, and crusting on atrophic skin that eventually result in cicatricial alopecia.
1
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Erosive pustular dermatosis of the scalp | |
| fr | Dermatose pustuleuse érosive du cuir chevelu | |
| de | Erosiv-pustulöse Dermatose der Kopfhaut | |
| es | Dermatosis pustulosa erosiva de cuero cabelludo | |
| it | Dermatosi pustolosa erosiva del cuoio capelluto | |
| nl | Erosieve pustuleuze dermatose van schedelhuid | |
| pt | Dermatose pustular erosiva do couro cabeludo | |
| pl | Nadżerkowe krostkowe zapalenie skóry głowy | |
| cs | Erozivní pustulární dermatóza skalpu | |
| uk | Ерозивно-пустульозний дерматоз волосистої частини голови | |
| zh | 头皮糜烂性脓疱性皮病 | |
| tr | Kafa derisinin erozif püstüler dermatozu |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L98.8 | NTBT | attributed | validated |
| ICD-11 | EG30.1 | E | specific | validated |
| MONDO | 0016379 | E | validated | |
| UMLS | C0406464 | E | validated | |
| GARD | 2188 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
3
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.
5
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.
6
HPO phenotypes
5 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0001595 | Abnormality of the hair | |
| HP:0010783 | Erythema | |
| HP:0200039 | Pustule | |
| Frequent (79-30%) | ||
| HP:0004552 | Scarring alopecia of scalp | |
| HP:0200041 | Skin erosion | |
Run the differential on the 3 obligate / very frequent terms
7
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/222
- This record as JSON: /data/d/222.json