Atopic Keratoconjunctivitis in Children: Clinical Features and Diagnosis
Authors: Dominique Brémond-Gignac, Ken K. Nischal, Bruno Mortemousque, Eva Gajdosova, David B. Granet, Frédéric Chiambaretta
Journal: Ophthalmology (2015)

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.1Calonge M. Herreras J.M. Clinical grading of atopic keratonconjunctivitis.Curr Opin Allergy Clin Immunol. 2007; 7: 442-445Crossref PubMed Scopus (30) Google Scholar 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, Dennie–Morgan folds of the lower lid and blepharitis were present in ≥65% of patients. Other clinical features such as giant papillae (>1 mm diameter), Dennie–Morgan double folds of the upper lid, pseudoptosis, infiltration of the inferior conjunctiva, Horner–Trantas dots, and madarosis were present in ≥39% of patients.Table 1Clinical Features of Patients (n = 23) with Atopic KeratoconjunctivitisClinical FeaturesPresent, n (%)Conjunctival hyperemia22 (96)Follicles19 (83)Papillae upper/lower19 (83)Giant papillae (> 1 mm diameter)14 (61)Horner–Trantas dots (limbus)9 (39)Swelling of the limbus9 (39)Chemosis (conjunctival)6 (26)Limbal—neovascularization/corneal opacification11 (48)Keratitis (superficial punctate keratitis, shield ulcer, ulcerations and corneal erosions)20 (87)Infiltration of inferior conjunctiva12 (52)Dennie–Morgan Double fold lower lid18 (78) Double fold upper lid13 (57)Pseudoptosis12 (52)Facial cutaneous fissures (ears and canthus)15 (65)Anterior blepharitis15 (65)Posterior blepharitis15 (65)Eczema, thickened and dry eyelid22 (96)Thickened and dry skin Facial20 (87) Body19 (83)Madarosis10 (44) Open table in a new tab In this report, AKC in children is defined 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 Horner–Trantas dots and giant papillae. The clinical features described here may be used to define a grading system for the identification 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.2Bremond-Gignac D. Donadieu J. Leonardi A. et al.Prevalence of vernal keratoconjunctivitis: a rare disease?.Br J Ophthalmol. 2008; 92: 1097-1102Crossref PubMed Scopus (84) Google Scholar Vernal keratoconjunctivitis generally ends at puberty; however, in some cases, it is thought that VKC may "evolve" into AKC in adulthood.2Bremond-Gignac D. Donadieu J. Leonardi A. et al.Prevalence of vernal keratoconjunctivitis: a rare disease?.Br J Ophthalmol. 2008; 92: 1097-1102Crossref PubMed Scopus (84) Google Scholar In the absence of typical clinical signs of VKC, a child with atopic dermatitis may be diagnosed with AKC and not VKC.1Calonge M. Herreras J.M. Clinical grading of atopic keratonconjunctivitis.Curr Opin Allergy Clin Immunol. 2007; 7: 442-445Crossref PubMed Scopus (30) Google Scholar Vernal keratoconjunctivitis presents with highly specific symptoms, such as photophobia, tearing, pseudoptosis, thick mucus discharge, and shield ulcers.2Bremond-Gignac D. Donadieu J. Leonardi A. et al.Prevalence of vernal keratoconjunctivitis: a rare disease?.Br J Ophthalmol. 2008; 92: 1097-1102Crossref PubMed Scopus (84) Google Scholar Children with VKC may present with atopic dermatitis; however, it is not a prerequisite for diagnosis.1Calonge M. Herreras J.M. Clinical grading of atopic keratonconjunctivitis.Curr Opin Allergy Clin Immunol. 2007; 7: 442-445Crossref PubMed Scopus (30) Google Scholar In contrast, evidence of atopic dermatitis must be present for a diagnosis of AKC to be made.1Calonge M. Herreras J.M. Clinical grading of atopic keratonconjunctivitis.Curr Opin Allergy Clin Immunol. 2007; 7: 442-445Crossref PubMed Scopus (30) Google Scholar In accordance with the findings 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.3Belfort R. Marbeck P. Hsu C. et al.Epidemiological study of 134 subjects with allergic conjunctivitis.Acta Ophthalmol Scand. 2000; 78: 38-40Crossref Google Scholar In Japanese populations, VKC cases with any history of atopic dermatitis is diagnosed as AKC, regardless of patient age.4Ebihara 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: 365-372Crossref PubMed Scopus (85) Google Scholar In Europe, these cases would only be diagnosed as AKC if the symptoms continued past puberty and occurred concurrently with keratoconjunctivitis.1Calonge M. Herreras J.M. Clinical grading of atopic keratonconjunctivitis.Curr Opin Allergy Clin Immunol. 2007; 7: 442-445Crossref PubMed Scopus (30) Google Scholar 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 specific 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.5Erdinest N. Solomon A. Topical immunomodulators in the management of allergic eye diseases.Curr Opin Allergy Clin Immunol. 2014; 14: 457-463Crossref PubMed Scopus (21) Google Scholar 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. Download .pdf (.29 MB) Help with pdf files Figure 1