ORPHA:505
Graham Little-Piccardi-Lassueur syndrome
Synonyms: Graham Little syndrome · Piccardi-Lassueur-Little syndrome
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult
- Status
- active
A variant of lichen planopilaris characterized by the clinical triad of progressive cicatricial (scarring) alopecia of the scalp, follicular keratotic papules on glabrous skin, and variable alopecia of the axillae and groin.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Graham Little-Piccardi-Lassueur syndrome | Graham Little syndrome · Piccardi-Lassueur-Little syndrome |
| fr | Syndrome de Graham Little-Piccardi-Lassueur | Syndrome de Graham Little · Syndrome de Piccardi-Lassueur-Little |
| de | Graham Little-Piccardi-Lassueur-Syndrom | Picardi-Lassueur-Little-Syndrom |
| es | Síndrome de Graham Little-Piccardi-Lassueur | Síndrome de Graham Little · Síndrome de Piccardi-Lassueur-Little |
| it | Sindrome di Graham Little-Piccardi-Lassueur | Sindrome di Graham Little · Sindrome di Picardi-Lassueur-Little |
| nl | Syndroom van Graham Little-Piccardi-Lassueur | Syndroom van Graham Little · Syndroom van Piccardi-Lassueur-Little |
| pt | Síndrome Graham Little-Piccardi-Lassueur | Síndrome Graham Little · Síndrome Picardi-Lassueur-Little |
| pl | Zespół Grahama Little'a,Piccardi i Lassueura | Zespół Grahama Little'a · Zespół Piccardi, Lassueura i Little'a |
| cs | Graham Littleův-Piccardiho-Lassueurův syndrom | Graham Littleův syndrom · Piccardiho-Lassueurův-Littleův syndrom · Graham Little-Piccardi-Lassueurův syndrom · Piccardi-Lassueur-Littleův syndrom |
| uk | Синдром Грема Літтла-Піккарді-Лассюра | Синдром Грема Літтла · Синдром Піккарді-Лассюра-Літтла |
| zh | Graham Little-Piccardi-Lassueur综合征 | Piccardi-Lassueur-Little综合征 |
| tr | Graham Little-Piccardi-Lassueur sendromu | Graham Little sendromu · Piccardi-Lassueur-Little sendromu |
Definition in:
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Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L66.1 | NTBT | attributed | validated |
| ICD-11 | EA91.2 | NTBT | index | validated |
| MONDO | 0018858 | E | validated | |
| UMLS | C4273658 | E | validated | |
| GARD | 3195 | 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 (2):
- ORPHA:79364Alopecia
rare skin diseases - ORPHA:254370Rare cutaneous lichen planus
rare skin diseases
No children.
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Genes
1 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| HLA-DRA | Major susceptibility factor in | Not yet assessed | HGNC:4947 | ENSG00000204287 | 142860 | 6p21.32 | major histocompatibility complex, class II, DR alpha |
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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.
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HPO phenotypes
7 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0001596 | Alopecia | |
| HP:0007468 | Perifollicular hyperkeratosis | |
| HP:0002215 | Sparse axillary hair | |
| HP:0002225 | Sparse pubic hair | |
| HP:0002209 | Sparse scalp hair | |
| Frequent (79-30%) | ||
| HP:0100725 | Lichenification | |
| HP:0000989 | Pruritus | |
Run the differential on the 5 obligate / very frequent terms
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/505
- This record as JSON: /data/d/505.json