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The Frequency of Signs of Meibomian Gland Dysfunction in Children with Epidermolysis Bullosa

Sophie M Jones1, Kate A Smith2, Minakshi Jain2

  • 1Clinical and Academic Department of Ophthalmology, Great Ormond Street Hospital for Children, London, United Kingdom; Developmental Biology Unit, Institute of Child Health, University College London, London, United Kingdom.

Ophthalmology
|February 16, 2016
PubMed

Insights

Most children with epidermolysis bullosa (EB) show signs of meibomian gland dysfunction (MGD). This study highlights the high prevalence of MGD in pediatric EB patients, offering insights into ocular surface issues.

Area of Science:

  • Ophthalmology
  • Genetics
  • Dermatology

Background:

  • Epidermolysis bullosa (EB) is a group of rare genetic disorders characterized by extreme skin fragility.
  • Ocular surface complications are common in EB, but the specific prevalence of meibomian gland dysfunction (MGD) remains understudied in pediatric populations.

Purpose of the Study:

  • To investigate the frequency of meibomian gland dysfunction (MGD) in children diagnosed with various forms of epidermolysis bullosa (EB).
  • To establish a baseline understanding of MGD prevalence in pediatric EB patients to better explain associated ocular surface anomalies.

Main Methods:

  • A hospital-based, cross-sectional study was conducted over seventeen months.
  • Prospective ophthalmic examinations were performed on 105 children with EB, including a standardized assessment of meibomian gland function.
  • Participants represented diverse EB subtypes: junctional EB, simplex EB, autosomal recessive dystrophic EB, and autosomal dominant dystrophic EB.

Main Results:

  • Out of 105 children with EB, 92 (87.62%) presented with at least one sign of meibomian gland dysfunction (MGD).
  • The study included children with a mean age of 7.42 years, covering a wide age range from under one month to over 17 years.
  • The participants comprised 8.6% with junctional EB, 34.3% with simplex EB, 34.3% with autosomal recessive dystrophic EB, and 22.9% with autosomal dominant dystrophic EB.

Conclusions:

  • The majority of children with epidermolysis bullosa (EB) exhibit signs of meibomian gland dysfunction (MGD).
  • This research provides the first prospective ocular surface evaluation in pediatric EB patients using a recognized classification for lid margin assessment.
  • The high prevalence of MGD in this cohort may contribute to understanding the ocular surface abnormalities observed in children with EB.
Abstract

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