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Childhood blepharokeratoconjunctivitis: characterising a severe phenotype in white adolescents
  1. Samer Hamada1,2,
  2. Imran Khan1,2,
  3. Alastair K Denniston1,2,
  4. Saaeha Rauz1,2
  1. 1Academic Unit of Ophthalmology, School of Immunity and Infection, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK
  2. 2Centre for Translational Inflammation Research, University of Birmingham Research Laboratories, Queen Elizabeth Hospital Birmingham, Birmingham, UK
  1. Correspondence to Dr Saaeha Rauz, Academic Unit of Ophthalmology, School of Immunity and Infection, College of Medical and Dental Sciences, University of Birmingham, Birmingham and Midland Eye Centre, Dudley Road, Birmingham B18 7QU, UK; s.rauz{at}bham.ac.uk

Abstract

Background The syndrome of childhood blepharokeratoconjunctivitis (BKC) is frequently underestimated. While prevalent and aggressive among Indo-Pakistani/Middle-Eastern populations, we observe a recalcitrant destructive phenotype in white children/adolescents that persists into early adulthood and may require systemic immunosuppression.

Methods A cohort of 10 white patients (20 eyes), median age 15.2 (range 6–27) years were identified among 62 patients with BKC attending a tertiary referral centre. Clinical features were graded and lid/conjunctiva swabs were performed, before instituting a hierarchical therapeutic protocol comprising lid hygiene, topical/systemic antibiotics, intensive topical glucocorticoids and systemic immunosuppression.

Results The median duration of symptoms prior to presentation was 4.3 (range 1.2–16.3) years, with 14 eyes (nine patients) demonstrating 360° peripheral corneal vascularisation associated with encroachment/involvement of the visual axis in 10 eyes (six patients). Corneal perforation(s) occurred in three eyes (two patients). Intensive topical glucocorticoids enabled disease control in 10 eyes (seven patients). In six eyes (three patients), persistent active disease necessitated systemic immunosuppression (azathioprine (2), mycophenolate mofetil (1), prednisolone (1)) achieving disease remission within three months with no adverse events reported.

Conclusions Suboptimal treatment of BKC in white children may permit a progressively destructive sight-threatening phenotype, which may last into adulthood and require immunosuppression. Appropriate aggressive steroid-based and steroid-sparing strategies are vital for disease remission.

  • Corneal perforation
  • ethnicity
  • immunosuppression
  • ocular surface inflammation
  • cornea
  • ocular surface
  • immunology
  • clinical trial
  • inflammation

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Footnotes

  • SH and IK contributed equally to this work.

  • Funding The Academic Unit of Ophthalmology is supported by the Birmingham Eye Foundation (Registered (UK) Charity 257549).

  • Competing interests None.

  • Ethics approval Ethics approval was provided by 'Inflammation in Ocular Surface Disease' (LREC ref: 08/H1206/165; Birmingham East, North and Solihull Research Ethics Committee.).

  • Provenance and peer review Not commissioned; externally peer reviewed.

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