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Reports

Atopic Keratoconjunctivitis in
Children: Clinical Features and
Diagnosis
Distinguishing between vernal keratoconjunctivitis (VKC) and
atopic keratoconjunctivitis (AKC) can be challenging. Historically,
AKC is rarely recognized as a diagnostic entity before puberty and
is thought to occur predominantly in adults. If a young patient were
to present with AKC-like symptoms and atopic dermatitis, they
might be diagnosed with VKC.1 The aim of this report was to
establish guidelines for distinguishing diagnosis between AKC
and VKC.
We conducted a case survey of 23 pediatric patients with severe
keratoconjunctivitis and atopic dermatitis who presented at 4 centers between 2011 and 2013. Mean ages at onset of symptoms and
at initial presentation to an ophthalmologist were 5.2 and 8.1 years,
respectively. All patients suffered from eczema and conjunctivitis/
keratitis, and the majority (74%) had a family history of atopy and
were affected by asthma and allergic rhinitis. The clinical features
of patients with AKC are presented in Table 1 and Figure 1
(available at www.aaojournal.org). The most prevalent clinical
features within this study were conjunctival hyperemia and
eczema, both of which were reported in 96% of patients. Other
clinical features include follicles, keratitis, and thickened dry
skin, which were present in 83% of patients. Papillae,
DennieeMorgan folds of the lower lid and blepharitis were
present in 65% of patients. Other clinical features such as giant
papillae (>1 mm diameter), DennieeMorgan double folds of the
upper lid, pseudoptosis, inltration of the inferior conjunctiva,
HornereTrantas dots, and madarosis were present in 39% of
patients.
In this report, AKC in children is dened as the presence of
severe allergic conjunctivitis with atopic dermatitis that is diagnosed before 16 years of age. This may be accompanied by the
presence or absence of the following clinical features: conjunctival
hyperemia with eczema, madarosis, and blepharitis, with the
absence of HornereTrantas dots and giant papillae. The clinical
features described here may be used to dene a grading system for
the identication of AKC in children. No single clinical feature
viewed in isolation can accurately differentiate between AKC and
VKC.
Vernal keratoconjunctivitis is a rare, yet severe, form of allergic
conjunctivitis, estimated to affect 3.2 out of every 10 000 inhabitants in western Europe.2 Vernal keratoconjunctivitis generally
ends at puberty; however, in some cases, it is thought that VKC
may evolve into AKC in adulthood.2 In the absence of typical
clinical signs of VKC, a child with atopic dermatitis may be
diagnosed with AKC and not VKC.1 Vernal keratoconjunctivitis
presents with highly specic symptoms, such as photophobia,
tearing, pseudoptosis, thick mucus discharge, and shield ulcers.2
Children with VKC may present with atopic dermatitis; however,
it is not a prerequisite for diagnosis.1 In contrast, evidence of
atopic dermatitis must be present for a diagnosis of AKC to be
made.1

In accordance with the ndings reported herein, AKC in children may be more prevalent than initially believed; in an epidemiologic study of 134 patients with allergic conjunctivitis, 55% of
patients with AKC reported an onset of symptoms before 10 years
of age.3 In Japanese populations, VKC cases with any history of
atopic dermatitis is diagnosed as AKC, regardless of patient
age.4 In Europe, these cases would only be diagnosed as AKC if
the symptoms continued past puberty and occurred concurrently
with keratoconjunctivitis.1 The multifactorial assessment of key
clinical signs presented, taking into consideration the presence of
AKC-related clinical features and the absence of VKC-related
clinical features, in combination with a history of eczema and
conjunctivitis/keratitis, may promote accurate diagnosis of AKC in
children. Atopic keratoconjunctivitis and VKC differ in relation to
specic treatment needs. The dermatologic manifestations of AKC
need to be treated with emollients and demulcents and, if necessary, corticosteroids. Furthermore, although immunomodulators
such as cyclosporine and tacrolimus are effective treatments for
both severe AKC and VKC, lower concentrations of these drugs
may be required in patients with AKC.5 These differences in the
optimal treatment of AKC and VKC highlight the importance of
early and accurate diagnosis, in informing effective treatment
strategies and improving patient outcomes.

DOMINIQUE BRMOND-GIGNAC, MD, PHD1,2


KEN K. NISCHAL, MD, PHD3,4
BRUNO MORTEMOUSQUE, MD, PHD5
EVA GAJDOSOVA, MD, PHD3
DAVID B. GRANET, MD, PHD6
FRDRIC CHIAMBARETTA, MD, PHD7
1
Pediatric Ophthalmology Department, University Hospital NeckerEnfants Malades, Paris, France; 2CNRS Unit FR3636, Paris V
University, Paris, France; 3Clinical and Academic Department of
Ophthalmology, Great Ormond Street Hospital for Children, London,
UK; 4Pediatric Ophthalmology, Strabismus and Adult Motility UPMC
Eye Center, Childrens Hospital of Pittsburgh, Pittsburgh,
Pennsylvania; 5Ophthalmology Department, University Hospital of
Pontchaillou, Rennes, France; 6Ratner Childrens Eye Center and
Shiley Eye Center, University of California, San Diego, California;
7
Ophthalmology Department, University Hospital Gabriel Montpied,
Clermont-Ferrand, France

Financial Disclosure(s): The authors have made the following disclosures: Allergan, Inc. (Irvine, CA) funded medical writing support but
had no role in the design or conduct of this research.
Author Contributions:
Conception and design: Brmond-Gignac, Nischal, Mortemousque,
Gajdosova, Granet, Chiambaretta
Analysis and interpretation: Brmond-Gignac, Nischal, Mortemousque,
Gajdosova, Granet, Chiambaretta
Data collection: Brmond-Gignac, Nischal, Mortemousque, Gajdosova,
Granet, Chiambaretta
Overall responsibility: Brmond-Gignac, Nischal, Mortemousque,
Gajdosova, Granet, Chiambaretta

Ophthalmology Volume -, Number -, Month 2015


Table 1. Clinical Features of Patients (n 23) with Atopic
Keratoconjunctivitis
Clinical Features
Conjunctival hyperemia
Follicles
Papillae upper/lower
Giant papillae (> 1 mm diameter)
HornereTrantas dots (limbus)
Swelling of the limbus
Chemosis (conjunctival)
Limbaldneovascularization/corneal opacication
Keratitis (supercial punctate keratitis, shield ulcer,
ulcerations and corneal erosions)
Inltration of inferior conjunctiva
DennieeMorgan
Double fold lower lid
Double fold upper lid
Pseudoptosis
Facial cutaneous ssures (ears and canthus)
Anterior blepharitis
Posterior blepharitis
Eczema, thickened and dry eyelid
Thickened and dry skin
Facial
Body
Madarosis

Present, n (%)
22
19
19
14
9
9
6
11
20

(96)
(83)
(83)
(61)
(39)
(39)
(26)
(48)
(87)

12 (52)
18
13
12
15
15
15
22

(78)
(57)
(52)
(65)
(65)
(65)
(96)

20 (87)
19 (83)
10 (44)

Correspondence:
Dominique Brmond-Gignac, Pediatric Ophthalmology Department,
AP-HP, University Hospital Necker-Enfants Malades, 149 Rue de
Svres, 75015 Paris, France. E-mail: dominique.bremond@nck.aphp.fr

References
1. Calonge M, Herreras JM. Clinical grading of atopic keratonconjunctivitis. Curr Opin Allergy Clin Immunol 2007;7:4425.
2. Bremond-Gignac D, Donadieu J, Leonardi A, et al. Prevalence
of vernal keratoconjunctivitis: a rare disease? Br J Ophthalmol
2008;92:1097102.
3. Belfort R, Marbeck P, Hsu C, et al. Epidemiological study of
134 subjects with allergic conjunctivitis. Acta Ophthalmol
Scand 2000;78:3840.
4. Ebihara N, Ohashi Y, Uchio E, et al. A large prospective
observational study of novel cyclosporine 0.1%
aqueous ophthalmic solution in the treatment of severe
allergic conjunctivitis. J Ocul Pharmacol Ther 2009;25:
36572.
5. Erdinest N, Solomon A. Topical immunomodulators in the
management of allergic eye diseases. Curr Opin Allergy Clin
Immunol 2014;14:45763.

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