raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:1221

Cheilitis glandularis

Type
Disease
Group
Disorder
Inheritance
Not applicable
Age of onset
Adolescent, Adult, Childhood, Elderly
Status
active

A rare skin disease of unknown origin characterized by macrocheilia and secretions of thick saliva from swollen labial minor salivary glands.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enCheilitis glandularis
frChéilite glandulaire
deCheilitis glandularisCheilitis glandularis apostematosa · Von-Baelz-Krankheit
esQueilitis glandular
itCheilite ghiandolare
nlCheilitis glandularis
ptQueilite glandular
plZapalenie gruczołowe warg
csGlandulární cheilitida
ukЗалозистий хейліт
zh腺性唇炎
trCheilitis glandularis

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
OMIM118330Evalidated
ICD-10K13.0NTBTinclusionvalidated
ICD-11DA00.0NTBTindexvalidated
MONDO0007313Evalidated
MeSHC535921Evalidated
UMLSC0267034Evalidated
GARD412Evalidated

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):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

2 records
TypeClassValueAreaQualificationValidationSource
Point prevalence<1 / 1 000 000—WorldwideClass onlyValidatedORPHANET
Cases/families100 casesWorldwideCase(s)Validated[EXPERT]

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
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0010286Abnormal salivary gland morphology
HP:0010978Abnormality of immune system physiology
HP:0002664Neoplasm
HP:0002860Squamous cell carcinoma
HP:0000179Thick lower lip vermilion

Run the differential on the 5 obligate / very frequent terms

7

Sources for this card