The eye in epidermolysis bullosa
- L Tonga,
- P R Hodgkinsa,
- J Denyerb,
- D Brosnahana,
- J Harperb,
- I Russell-Eggitta,
- D S I Taylora,
- D Athertonb
- aDepartment of Ophthalmology, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London WC1N 3JH, bDepartment of Dermatology, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London WC1N 3JH
- Mr P Hodgkins, Southampton Eye Unit, Tremona Road, Southampton, SO16 6YD.
- Accepted 11 September 1998
Abstract
AIMS To describe the ophthalmic findings in a large cohort of epidermolysis bullosa (EB) patients managed in one large specialist centre.
METHODS A case note review of consecutive patients seen at Great Ormond Street Children’s Hospital. Data on the dermatological disease, ophthalmic history, and examination were collected and coded onto a data sheet.
RESULTS 181 patients: 50 (28%) simplex EB; 15 (8%) junctional EB; 28 (15%) autosomal dominant dystrophic EB; 72 (40%) autosomal recessive dystrophic EB; nine patients (5%) with dystrophic EB whose inheritance could not be ascertained; and seven cases (4%) of EB that could not be classified. Ocular problems were found in 12% (n=6) of simplex patients and 40% (n=6) of those with junctional disease. One patient (of 28) in the autosomal dominant dystrophic group had ocular involvement and 51% (37/72) of patients in the autosomal recessive dystrophic group had ophthalmic complications: corneal (25/72), lid ectropions (3/72), lid blisters (5/72), and symblepharon (3/72).
CONCLUSION Ophthalmic complications are common in EB overall but the incidence varies widely with subtype. Ophthalmic complications are the most severe in the dystrophic recessive and junctional subtypes where there is a need for extra vigilance. The major treatment modality was use of ocular lubricants.
