raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:687730

Unifocal Langerhans cell histiocytosis

Synonyms: Unifocal Langerhans cell granulomatosis · Unifocal histiocytosis X

Type
Clinical subtype
Group
Subtype of disorder
Inheritance
Not applicable
Status
active

Orphadata ships no English definition for this code.

1

Names in Orphadata languages

9 of 12 languages
LangNameSynonyms
enUnifocal Langerhans cell histiocytosisUnifocal Langerhans cell granulomatosis · Unifocal histiocytosis X
frHistiocytose langerhansienne unifocaleGranulomatose à cellules de Langerhans unifocale · Histiocytose X unifocale
deLangerhans-Zell-Histiozytose, unifokaleHistiozytose X, unifokale · Langerhans-Zell-Granulomatose, unifokale
esHistiocitosis de células de Langerhans unifocalGranulomatosis de células de Langerhans unifocal · Histiocitosis X unifocal
itUnifocal Langerhans cell histiocytosis
nlUnifocale langerhanscelhistiocytoseUnifocale histiocytose X · Unifocale langerhanscelgranulomatose
ptHistiocitose unifocal de células LangerhansGranulomatose unifocal de células Langerhans · Histiocitose X unifocal
plJednoogniskowa histiocytoza z komórek Langerhansa= en
csUnifokální histiocytóza z Langerhansových buněk= en
2

Cross-references

qualifier = Orphanet mapping relation

No cross-references in this release.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

  • ORPHA:389Langerhans cell histiocytosis
    rare respiratory diseases; rare hematological diseases; rare systemic and rheumatological diseases; rare neoplastic diseases; rare transplant-related disorders

No children.

4

Genes

1 direct
GeneAssociationStatusHGNCEnsemblOMIMLocusNameSource of validation
BRAFDisease-causing somatic mutation(s) inAssessedHGNC:1097ENSG000001577641647577q34B-Raf proto-oncogene, serine/threonine kinase22996177[PMID]
5

Prevalence and incidence

0 records

No prevalence record in this release.

6

HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

7

Sources for this card