Seborrhea-like dermatitis with psoriasiform elements
- Type
- Disease
- Group
- Disorder
- Inheritance
- Autosomal dominant
- Age of onset
- Childhood
- Status
- active
A rare, genetic, epidermal disorder characterized by a chronic, diffuse, fine, scaly erythematous rash on the face (predominantly the chin, nasolabial folds, eyebrows), around the earlobes and over the scalp, associated with hyperkeratosis over elbows, knees, palms, soles and metacarpophalangeal joints, in the absence of associated rheumatological or neurological disorders. Cold weather, emotional stress and strenuous physical activity may exacerbate symptoms.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Seborrhea-like dermatitis with psoriasiform elements | |
| fr | Dermatite séborrhéïque-like avec des éléments psoriasiques | |
| de | Seborrhö-ähnliche Dermatitis mit psoriasiformen Ausschlägen | |
| es | Dermatitis tipo seborreica con elementos psoriasiformes | |
| it | Dermatite simil-seborroica con elementi psoriasiformi | |
| nl | Seborroe-achtige dermatitis met psoriasiforme elementen | |
| pt | Dermatite seborreica-like com elementos psoriasiformes | |
| pl | Zapalenie skóry podobne do łojotokowego zapalenia skóry z elementami łuszczycopodobnymi | |
| cs | Seborrhea-like dermatitida s psoriaziformními prvky | |
| uk | Себорейний дерматит з псоріазоподібними елементами | |
| zh | 脂溢性皮炎样皮炎伴银屑病样成分 | |
| tr | Psoriasiform elementler ile seyreden sebore benzeri dermatit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 610227 | E | validated | |
| ICD-10 | L21.8 | NTBT | attributed | validated |
| ICD-11 | EA81.0 | NTBT | attributed | validated |
| ICD-11 | EA81.1 | NTBT | attributed | validated |
| ICD-11 | EA81.Y | NTBT | attributed | validated |
| MONDO | 0012446 | E | validated | |
| MeSH | C565217 | E | validated | |
| UMLS | C1853258 | 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:183426Genetic epidermal disorder
rare genetic diseases - ORPHA:79359Other epidermal disorder
rare skin diseases
No children.
Genes
1 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| ZNF750 | Disease-causing germline mutation(s) in | Assessed | HGNC:25843 | ENSG00000141579 | 610226 | 17q25.3 | zinc finger protein 750 | 16751772[PMID] |
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 44 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.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/168606
- This record as JSON: /data/d/168606.json