Vernal keratoconjunctivitis
Synonyms: Spring catarrh
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Childhood
- Status
- active
A rare disorder of the anterior segment of the eye, characterized by a severe recurrent allergic reaction affecting the cornea and the conjunctiva. It presents with red eyes, ocular itching, photophobia, foreign body sensation, mucous discharge, blepharospasm, and blurring of vision. The symptoms are typically bilateral but may be asymmetric. Characteristic signs include conjunctival injection, giant papillae mostly on the upper tarsal conjunctiva (cobblestone appearance), limbal gelatinous infiltrates (Horner-Trantas dots), and variable corneal signs. The condition is more prevalent in hot climates and most commonly affects young boys.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Vernal keratoconjunctivitis | Spring catarrh |
| fr | Kératoconjonctivite vernale | Kératoconjonctivite printanière |
| de | Keratokonjunctivitis vernalis | Frühjahrskonjunktivitis · Frühlingskatarrh · VKC |
| es | Queratoconjuntivitis primaveral | Catarro primaveral |
| it | Cheratocongiuntivite primaverile | Cheratocongiuntivite Vernal |
| nl | Vernale keratoconjunctivitis | Lente-catarre |
| pt | Queratoconjuntivite primaveril | Catarro primaveril |
| pl | Wiosenne zapalenie rogówki i spojówki | = en |
| cs | Vernální keratokonjunktivitida | Jarní katar · Vernální katar, VKC |
| uk | Весняний кератоконюнктивіт | Весняний катар |
| zh | 春季角结膜炎 | 春季结膜炎 |
| tr | Vernal keratokonjunktivit | Bahar nezlesi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | H16.2 | NTBT | attributed | validated |
| ICD-11 | 9A60.5 | E | specific | validated |
| MONDO | 0019085 | E | validated | |
| UMLS | C0022577 | E | validated | |
| GARD | 7854 | E | validated | |
| MedDRA | 10081000 | 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:519280Rare conjunctivitis
rare ophthalmic disorders - ORPHA:98623Syndromic keratoconus
rare ophthalmic disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
7 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | >1 / 1000 | 151 / 100 000 | Italy | Value and class | Validated | 18356259[PMID] |
| Point prevalence | 1-5 / 10 000 | 45.5 / 100 000 | Finland | Value and class | Validated | 18356259[PMID] |
| Point prevalence | 1-5 / 10 000 | 49.5 / 100 000 | Sweden | Value and class | Validated | 18356259[PMID] |
| Point prevalence | 1-5 / 10 000 | 26 / 100 000 | Netherlands | Value and class | Validated | 18356259[PMID] |
| Point prevalence | 1-5 / 10 000 | 20 / 100 000 | France | Value and class | Validated | 18356259[PMID] |
| Point prevalence | 1-5 / 10 000 | 11 / 100 000 | Norway | Value and class | Validated | 18356259[PMID] |
| Point prevalence | 1-5 / 10 000 | 32 / 100 000 | Europe | Value and class | Not yet validated | 18356259[PMID] |
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
23 annotations · validated 2024-09-20| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0000502 | Abnormal conjunctiva morphology | |
| HP:0000481 | Abnormal cornea morphology | |
| HP:0000591 | Abnormal sclera morphology | |
| HP:0007879 | Allergic conjunctivitis | |
| HP:0012393 | Allergy | |
| HP:0030953 | Conjunctival hyperemia | |
| HP:0011496 | Corneal neovascularization | |
| HP:0009926 | Epiphora | |
| HP:0001096 | Keratoconjunctivitis | |
| HP:0000613 | Photophobia | |
| HP:0011859 | Punctate keratitis | |
| HP:0025337 | Red eye | |
| Frequent (79-30%) | ||
| HP:0000643 | Blepharospasm | |
| HP:0000622 | Blurred vision | |
| HP:0034804 | Corneal foreign body sensation | |
| HP:0025350 | Giant conjunctival papillae | |
| HP:0033841 | Ocular pruritus | |
| HP:0100699 | Scarring | |
| Occasional (29-5%) | ||
| HP:0000498 | Blepharitis | |
| HP:0012804 | Corneal ulceration | |
| HP:0000563 | Keratoconus | |
| HP:0007663 | Reduced visual acuity | |
| Very rare (<4-1%) | ||
| HP:0032107 | Limbal stem cell deficiency | |
Run the differential on the 12 obligate / very frequent terms
Annotation source: 10857837[PMID]_35015458[PMID]_30550738[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/70476
- This record as JSON: /data/d/70476.json