raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:163934

Atopic keratoconjunctivitis

Type
Disease
Group
Disorder
Inheritance
Not applicable
Age of onset
All ages
Status
active

A rare, chronic allergic disease of the cornea and conjunctiva occurring in all age groups, characterized by severe itching and burning sensation, conjunctival injection, photophobia and edema with serious cases leading to ulceration of the cornea which can result in blindness. It is often associated with atopic dermatitis.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enAtopic keratoconjunctivitis
frKératoconjonctivite atopique
deKeratokonjunktivitis, atopische
esQueratoconjuntivitis atópica
itCheratocongiuntivite atopica
nlAtopische keratoconjunctivitis
ptQueratoconjuntivite atópica
plAtopowe zapalenie spojówek i rogówki
csAtopická keratokonjunktivitida
ukАтопічний кератоконюнктивіт
zh特应性角膜结膜炎
trAtopik keratokonjunktivit

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10H16.2NTBTattributedvalidated
ICD-119A60.0YNTBTindexvalidated
MONDO0015599Evalidated
UMLSC1274788Evalidated
MedDRA10069664Evalidated

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

1 record
TypeClassValueAreaQualificationValidationSource
Point prevalence1-5 / 10 00015 / 100 000EuropeValue and classValidatedEuropean Medicines Agency 2009[OTHER]

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

15 annotations · validated 2023-09-12
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0007957Corneal opacity
HP:0000958Dry skin
HP:0000491Keratitis
HP:0001097Keratoconjunctivitis sicca
Frequent (79-30%)
HP:0000492Abnormal eyelid morphology
HP:0007879Allergic conjunctivitis
HP:0000498Blepharitis
HP:0000622Blurred vision
HP:0030953Conjunctival hyperemia
HP:0011496Corneal neovascularization
HP:0011457Loss of eyelashes
HP:0200026Ocular pain
HP:0000613Photophobia
Occasional (29-5%)
HP:0012375Chemosis
HP:0000559Corneal scarring

Run the differential on the 4 obligate / very frequent terms

Annotation source: 26278858[PMID]_28846256[PMID]

7

Sources for this card