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

Published:February 12, 2016DOI:


      To determine the frequency of meibomian gland dysfunction (MGD) in children with epidermolysis bullosa (EB).


      Hospital-based cross-sectional study.


      One hundred five children with different forms of EB.


      Prospective ophthalmic examination of children with EB presenting over seventeen months including meibomian gland assessment using a recognized classification.

      Main Outcome Measures

      Frequency of MGD.


      One hundred five children were recruited, 8.6% with junctional EB, 34.3% with simplex EB, 34.3% with autosomal recessive dystrophic EB, and 22.9% autosomal dominant dystrophic EB. Mean age was 7.42 years (range, 0.08–17.75 years). Ninety-two children (87.62%) demonstrated 1 or more features of MGD.


      Most children with EB exhibit signs of MGD. To the best of our knowledge, this is the first prospective ocular surface evaluation in children with EB to include lid margin evaluation using a recognized classification system. Our findings help explain some of the ocular surface anomalies seen in children with EB.

      Abbreviations and Acronyms:

      DDEB (dominant dystrophic epidermolysis bullosa), EB (epidermolysis bullosa), EBS (epidermolysis bullosa simplex), JEB (junctional epidermolysis bullosa), MGD (meibomian gland dysfunction), RDEB (recessive dystrophic epidermolysis bullosa)
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        • Fine J.D.
        • Eady R.A.J.
        • Bauer E.A.
        • et al.
        The classification of inherited epidermolysis bullosa (EB): report of the Third International Consensus Meeting on Diagnosis and Classification of EB.
        J Am Acad Dermatol. 2008; 58: 931-950
        • Tong L.
        • Hodgkins P.R.
        • Denyer J.
        • et al.
        The eye in epidermolysis bullosa.
        Br J Ophthalmol. 1999; 83: 323
        • Lin A.N.
        • Murphy F.
        • Brodie S.E.
        • Carter D.M.
        Review of ophthalmic findings in 204 patients with epidermolysis bullosa.
        Am J Ophthalmol. 1994; 118: 384-390
        • Camisa C.
        • Meisler D.M.
        Immunobullous diseases with ocular involvement.
        Dermatol Clin. 1992; 10: 555-570
        • McDonnell P.J.
        • Schofield O.M.
        • Spalton D.J.
        • Eady R.A.
        Eye involvement in junctional epidermolysis bullosa.
        Arch Ophthalmol. 1989; 107: 1635-1637
        • Zierhut M.
        • Thiel H.J.
        • Weidle E.G.
        • et al.
        Ocular involvement in epidermolysis bullosa acquisita.
        Arch Ophthalmol. 1989; 107: 398-401
        • McDonnell P.J.
        • Spalton D.J.
        The ocular signs and complications of epidermolysis bullosa.
        J R Soc Med. 1988; 81: 576-578
        • Fine J.D.
        • Johnson L.B.
        • Weiner M.
        • et al.
        Eye involvement in inherited epidermolysis bullosa: experience of the National Epidermolysis Bullosa Registry.
        Am J Ophthalmol. 2004; 138: 254
        • McDonnell P.J.
        • Schofield O.M.
        • Spalton D.J.
        • et al.
        The eye in dystrophic epidermolysis bullosa: clinical and immunopathological findings.
        Eye. 1989; 3: 79-83
        • Nelson J.D.
        • Shimazaki J.
        • Benitez-del-Castillo J.M.
        • et al.
        The International Workshop on Meibomian gland dysfunction: report of the Definition and Classification Subcommittee.
        Invest Ophthalmol Vis Sci. 2011; 52: 1930-1937
        • Bron A.J.
        • Benjamin L.
        • Snibson G.R.
        Meibomian gland disease. Classification and grading of lid changes.
        Eye. 1991; 5: 395-411
        • Tomlinson A.
        • Bron A.J.
        • Korb D.R.
        • et al.
        The International Workshop on Meibomian Gland Dysfunction: report of the Diagnosis Subcommittee.
        Invest Ophthalmol Vis Sci. 2011; 52: 2006-2049
        • Gans L.A.
        Eye lesions of epidermolysis bullosa. Clinical features, management, and prognosis.
        Arch Dermatol. 1988; 124: 762-764
        • Adamis A.P.
        • Schein O.D.
        • Kenyon K.R.
        Anterior corneal disease of epidermolysis bullosa simplex.
        Arch Ophthalmol. 1993; 111: 499
        • Mathers W.
        • Sachdev M.S.
        • Petroll W.M.
        • Jester J.V.
        Meibomian gland dysfunction in chronic blepharitis.
        Cornea. 1991; 10: 277-285
        • Snell R.
        • Lemp M.
        Clinical Anatomy of the Eye.
        Blackwell Science, Oxford, UK1998
        • Fine J.D.
        • Johnson L.B.
        • Suchindran C.
        The epidemiology of inherited epidermolysis bullosa: findings in the US, Canadian and European study populations.
        in: Fine J.D. Bauer E.A. Mcquire J. Moshell A. Clinical, Epidemiologic and Laboratory Advances and the Findings of the National EB Registry. Johns Hopkins University Press, Baltimore1999: 101-113
        • Bruckner-Tuderman L.
        Epidermolysis bullosa.
        Eur J Dermatol. 2008; 18: 214-216
        • McGrath J.A.
        • Mellerio J.E.
        Epidermolysis bullosa.
        Br J Hosp Med (Lond). 2006; 67: 188-191
        • Baudolin C.
        • Bron A.J.
        • Dogru M.
        • et al.
        2007 report of the Dry Eye Workshop.
        Ocular Surface. 2007; 5: 65-204
        • BenEzra D.
        Blepharitis and Conjunctivitis: Guideline for Diagnosis and Treatment.
        Editorial Glosa, 2006
        • Matsumoto Y.
        • Dogru M.
        • Tsubota K.
        Ocular surface findings in Hallpeau-Siemens subtype of dystrophic epidermolysis bullosa: report of a case and literature review.
        Cornea. 2005; 24: 474-479
        • Knop E.
        • Knop N.
        • Millar T.
        • et al.
        The International Workshop on Meibomian Gland Dysfunction: report of the Subcommittee on Anatomy, Physiology, and Pathophysiology of the Meibomian Gland.
        Invest Ophthalmol Vis Sci. 2011; 52: 1938-1978
        • Qiao J.
        • Yan X.
        Emerging treatment options for meibomian gland dysfunction.
        Clin Ophthalmol. 2013; 7: 1797-1803
        • Qazi Y.
        • Wong G.
        • Monson B.
        • et al.
        Corneal transparency: genesis, maintenance and dysfunction.
        Brain Res Bull. 2010; 81: 198-210