Vernal keratoconjunctivitis

Summary

Vernal keratoconjunctivitis (MONDO:0019085) is a disease and 15 clinical trials. Top therapeutic interventions include cyclosporine, betamethasone, and dexamethasone. A subtype of eye disorder — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: >1 / 1000 (Italy) [Orphanet-validated]
• Phenotypes (HPO): 23
• Clinical trials: 15

Clinical features

Epidemiology

Prevalence records

7 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Point prevalence	>1 / 1000	151	Italy	Validated
Point prevalence	1-5 / 10 000	45.5	Finland	Validated
Point prevalence	1-5 / 10 000	49.5	Sweden	Validated
Point prevalence	1-5 / 10 000	26	Netherlands	Validated
Point prevalence	1-5 / 10 000	20	France	Validated
Point prevalence	1-5 / 10 000	11	Norway	Validated
Point prevalence	1-5 / 10 000	32	Europe	Not yet validated

Signs & symptoms

Clinical features (HPO)

23 HPO clinical features (Orphanet curated; top 23 by frequency):

HPO ID	Term	Frequency
HP:0000481	Abnormal cornea morphology	Very frequent (80-99%)
HP:0000502	Abnormal conjunctiva morphology	Very frequent (80-99%)
HP:0000591	Abnormal sclera morphology	Very frequent (80-99%)
HP:0000613	Photophobia	Very frequent (80-99%)
HP:0001096	Keratoconjunctivitis	Very frequent (80-99%)
HP:0007879	Allergic conjunctivitis	Very frequent (80-99%)
HP:0009926	Epiphora	Very frequent (80-99%)
HP:0011496	Corneal neovascularization	Very frequent (80-99%)
HP:0011859	Punctate keratitis	Very frequent (80-99%)
HP:0012393	Allergy	Very frequent (80-99%)
HP:0025337	Red eye	Very frequent (80-99%)
HP:0030953	Conjunctival hyperemia	Very frequent (80-99%)
HP:0000622	Blurred vision	Frequent (30-79%)
HP:0000643	Blepharospasm	Frequent (30-79%)
HP:0025350	Giant conjunctival papillae	Frequent (30-79%)
HP:0033841	Ocular pruritus	Frequent (30-79%)
HP:0034804	Corneal foreign body sensation	Frequent (30-79%)
HP:0100699	Scarring	Frequent (30-79%)
HP:0000498	Blepharitis	Occasional (5-29%)
HP:0000563	Keratoconus	Occasional (5-29%)
HP:0007663	Reduced visual acuity	Occasional (5-29%)
HP:0012804	Corneal ulceration	Occasional (5-29%)
HP:0032107	Limbal stem cell deficiency	Very rare (<1-4%)

Identifiers

Disease identifiers

Field	Value
Canonical name	vernal keratoconjunctivitis
Mondo ID	MONDO:0019085
Orphanet	70476
ICD-11	670300288
SNOMED CT	317349009
UMLS	C0022577
MedGen	9622
GARD	0007854
NORD	1826
Is cancer (heuristic)	no

Also known as: Spring catarrh · Vernal Keratonconjunctivitis · VKC

Disease family

This is a subtype of eye disorder. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › disorder of orbital region › eye disorder › vernal keratoconjunctivitis

Related subtypes (119): ptosis, eye accommodation disease, corneal disorder, asthenopia, lens disorder, keratomalacia, scleral disorder, ocular siderosis, coloboma, luxation of globe, mucopolysaccharidosis type 1, lacrimal apparatus disorder, Foster-Kennedy syndrome, anterior dislocation of lens, uveal disorder, eyelid disorder, ocular hypotension, scotoma, exophthalmos, ophthalmia nodosa, eye degenerative disorder, refractive error, glaucoma, retinal disorder, eye allergy, ocular vascular disorder, optic neuritis, conjunctival disorder, ocular hypertension, Tietz syndrome, Alagille syndrome, glaucoma-sleep apnea syndrome, Marshall syndrome, microcornea-glaucoma-absent frontal sinuses syndrome, nail-patella syndrome, oculodentodigital dysplasia, piebaldism, Sturge-Weber syndrome, cerebrotendinous xanthomatosis, ocular cystinosis, alpha-mannosidosis, megalocornea-intellectual disability syndrome, mucolipidosis type IV, mucopolysaccharidosis type 6, Netherton syndrome, galactosialidosis, Niemann-Pick disease type A, ocular motor apraxia, Cogan type, Peters plus syndrome, isolated Pierre-Robin syndrome, ectodermal dysplasia-blindness syndrome, Sandhoff disease, SHORT syndrome, Sjogren-Larsson syndrome, Smith-Lemli-Opitz syndrome, Tay-Sachs disease, tyrosinemia type II, Ito hypomelanosis, X-linked cone dysfunction syndrome with myopia, red color blindness, oculocerebrorenal syndrome, Lowry-MacLean syndrome, pigment dispersion syndrome, hereditary hyperferritinemia with congenital cataracts, dyssegmental dysplasia-glaucoma syndrome, mevalonic aciduria, familial cavitary optic disk anomaly, blindness - scoliosis - arachnodactyly syndrome, fatty acyl-CoA reductase 1 deficiency, microcephaly-intellectual disability-sensorineural hearing loss-epilepsy-abnormal muscle tone syndrome, neurotrophic keratopathy, Cogan syndrome, atopic keratoconjunctivitis, rhizomelic chondrodysplasia punctata, Ehlers-Danlos syndrome, kyphoscoliotic type 1, IRVAN syndrome, Rothmund-Thomson syndrome type 2, microcornea-corectopia-macular hypoplasia syndrome, isolated anophthalmia-microphthalmia syndrome, Spasmus nutans, toxic maculopathy due to antimalarial drugs, syndromic recessive X-linked ichthyosis, acute zonal occult outer retinopathy, acute annular outer retinopathy, phakomatosis pigmentovascularis, lamellar ichthyosis, idiopathic linear interstitial keratitis, chondroectodermal dysplasia with night blindness, galactosemia, GM1 gangliosidosis, Gaucher disease, visual snow syndrome, extensive peripapillary myelinated nerve fibers, IgG4-related ophthalmic disorder, global developmental delay-visual anomalies-progressive cerebellar atrophy-truncal hypotonia syndrome, Gardner syndrome, anterior segment dysgenesis, isolated ankyloblepharon filiforme adnatum, hereditary optic neuropathy, essential strabismus, Axenfeld anomaly, eye neoplasm, isolated blepharochalasis, punctate inner choroidopathy, eye infectious disorder, vitreous body disorder, 9q33.3q34.11 microdeletion syndrome, autoimmune/inflammatory optic neuropathy, LTBP2-related ocular dysgenesis, ocular growth disorder, ocular dysgenesis caused by defects in PAX6 regulation, choroidal neovascularization, anterior segment developmental abnormality with extraocular manifestations, congenital optic disk excavation, neuroocular syndrome, isolated angioid streaks, multiple evanescent white dot syndrome, stellate multiform amelanotic choroidopathy, macular telangiectasia

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 15.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	6
PHASE4	3
PHASE3	3
PHASE1	2
PHASE2/PHASE3	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT01211327	PHASE4	COMPLETED	Topical Cyclosporine for Vernal Keratoconjunctivitis (VKC) in Rwanda
NCT02456025	PHASE4	UNKNOWN	Topical Tacrolimus in Vernal Keratoconjunctivitis
NCT03464435	PHASE4	COMPLETED	A Novel Combined Therapy for Refractory Vernal Keratoconjunctivitis
NCT06903884	PHASE3	RECRUITING	3% Diquafosol Ophthalmic Solution for Active Moderate-to-Severe Vernal Keratoconjunctivitis
NCT00426023	PHASE3	COMPLETED	Cyclosporin A Eye Drop Treatment in Vernal Keratoconjunctivitis
NCT00445120	PHASE2/PHASE3	COMPLETED	Lactobacillus Rhamnosus GG Oral Treatment Efficacy on Vernal Keratoconjunctivitis Treatment
NCT01751126	PHASE3	COMPLETED	Double-Masked Trial of NOVA22007 (1mg/mL Ciclosporin/Cyclosporine) Versus Vehicle in Pediatric Patients With Active Severe Vernal Keratoconjunctivitis
NCT03379311	PHASE1	COMPLETED	A Study of AK002 in Patients With Atopic Keratoconjunctivitis, Vernal Keratoconjunctivitis, and Perennial Allergic Conjunctivitis
NCT05353101	PHASE1	COMPLETED	Cyclosporine 0.05% Eye Drops for Vernal Keratoconjunctivitis Trial
NCT06913790	Not specified	ACTIVE_NOT_RECRUITING	Evaluation of Central Corneal Thickness in Patients With Vernal Keratoconjunctivitis Using Topical Steroids
NCT07138742	Not specified	NOT_YET_RECRUITING	Tacrolimus Eye Drops in Treatment of Vernal Keratoconjunctivitis
NCT02057822	Not specified	UNKNOWN	Cytokine Assay in Tears of Healthy Children and With Vernal Keratoconjunctivitis: Case Control Study and Monitoring of Cases at 6 Months
NCT03557203	Not specified	UNKNOWN	Dermatologic Tacrolimus Ointment on Eyelids in the Treatment of Refractory Vernal Keratoconjunctivitis and Atopic Keratoconjunctivitis
NCT04705584	Not specified	UNKNOWN	Topical Immuonosuppressant Drugs in Spring Catarrh
NCT05076084	Not specified	COMPLETED	Evaluation of the Efficacy and Safety of Tacrolimus Drops in Children With Vernal Keratoconjunctivitis

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
CYCLOSPORINE	4	3
BETAMETHASONE	4	1
DEXAMETHASONE	4	1
KETOTIFEN FUMARATE	4	1
LOTEPREDNOL ETABONATE	4	1
LIRENTELIMAB	3	1