External Ocular Manifestations in Autosomal Dominant Dystrophic Epidermolysis Bullosa; a Case Report
A 10-year-old girl was referred to Labbafinejad Medical Center with redness and bullous conjunctival lesions in her right eye. She had history of bullous and ulcerated itchy lesions on her trunk and palms since the age of two. The patient had also developed erythema and a bullous-like lesion in her right eye resulting in symblepharon formation and obliteration of the upper fornix following eye rubbing. She had previously twice undergone surgery to remove the symblepharon, however, each time it shortly relapsed with more severity.\nOn general examination, active and chronic bullous lesions were observed on the trunk and palms, which had led to scarring in some areas ( & ). She had dystrophic nails and absence of nails in some fingers (). There were also a number of milia on the back of her hands and hypertrophic scars on the upper and lower extremities (). The oral mucosa was normal and the patient did not have any swallowing complaints or other gastrointestinal disorders.\nOn ophthalmic examination she had bestcorrected visual acuity of 20/50 with +3.0−3.0 @140° in the right eye and uncorrected vision of 20/20 in the left. In the right eye, a symblepharon connected the medial portion of the upper lid to the upper nasal quadrant of the cornea resulting in limitation of eye movement especially downward and to the right ().Slitlamp biomicroscopy revealed mild subepithelial opacification in both corneas. The rest of the ophthalmic examination was unremarkable.