Case Report

Treatment Approach to Autosomal Recessive Dystrophic Epidermolysis Bullosa - Case Report

  • Rana Altan Yaycıoğlu
  • Yonca Aydın Akova
  • Fatma Selin Kaya
  • Sibel Oto

Received Date: 09.12.2009 Accepted Date: 09.02.2010 Turk J Ophthalmol 2010;40(2):107-112

Purpose:

To present the treatment approach to ocular complications in two cases with autosomal recessive dystrophic epidermolysis bullosa (EB) and to review the literature.

Material and Method:

Two teenage girls with EB, one with symblepharon (case 1) and the other with corneal opacity and vascularization (case 2) presented to our clinic. Case 1 had symblepharon, extending from the left upper eyelid to the superotemporal cornea, restricting the globe movements. During surgery, symblepharon lysis and lamellar keratectomy were followed by amniotic membrane transplantation to cover the cornea and palpebral conjunctiva. At presentation, case 2 was using artificial tears and topical cyclosporin A. The right eye had visual acuity of counting fingers, and opacity with superficial and deep vascularization in the lower third of the cornea. Topical bevacizumab and autologous serum were added to her treatment.

Results:

In Case 1, 63 months after surgery the eyelid margin was smooth and the eye was fully mobile. A mild symblepharon of the temporal conjunctiva in both eyes was present. Stromal thinning, superficial vascularization and opacification were significant in the temporal cornea. During the 8-month follow-up period of case 2, the visual acuity increased to 20/50. Slit-lamp examination showed a decrease in corneal opacity and vascularization.

Discussion:

According to our cases, alternative treatments such as amniotic membrane transplantation to treat symblepharon, and topical bevacizumab and autologous serum in intractable vascularization and opacity can be considered in cases of EB.

Keywords: Epidermolyzis bullosa, amniotic membrane, autologous serum, vascular endothelial growth factor, VEGF, bevacizumab

Full Text (Turkish)