Purpose To determine the frequency o f ocular manifesta-tions in inherited epidermolysis bullosa(EB)within the continental United States and to define the estimated cu-mulative risks of developing nonscarring(blisters or ero-sions)and scarring corneal manifestation s within each major EB subtype over time.Design Observational(cross-sectional and longitudinal).Methods Up to 16years of longitudinal follow-up was conducted on 3,280consec-utively enrolled patients in the National EB Registry,an epidemiologic study funded by the Na tional Institutes of Health.Data were stratified by majo r EB type and subtype.Frequencies of occurrence were dete rmined for eight vari-ables(corneal erosions or blistering;corneal scarring;symblepharons;blepharitis;ectro pions;lacrimal duct ob-struction;impaired vision;blindn ess)by contingency ta-bles,and cumulative risks were generated by life table analysis technique.Results The most common ocular manifestations were corneal erosio ns and blisters.Fre-quencies mirrored relative severity of skin disease,with74.10%of all patients with recessiv e dystrophic EB,Hallopeau-Siemens(RDEB-HS )and 47.50%of all pa-tients with junctional EB,Herlitz(JEB-H)experiencing at least one episode.Lower frequencie s were noted for corneal scarring.Symblepharons and ectrop ions were most com-monly seen in inversa RDEB and JEB-H,respectively.Blindness was reported in 6.47%of RD EB-HS patients.The cumulative risks of nonscarring and scarring corneal lesions in JEB-H at age 5are 83.18%an d 27.08%and at age 25are 83.18%and 72.22%.With tim e,the cumu-lative risk of each in RDEB-HS approached that reported in JEB-H patients.Conclusion Ocular d isease activity,par-ticularly corneal,is common in some EB subtypes.Careful ophthalmologic examination should become an integral part of the management of all patients wit h inherited EB. Purpose To determine the frequency of ocular manifesta- tions in inherited epidermolysis bullosa (EB) within the continental United States and to define the estimated cu-mulative risks of developing nonscarring (blisters or e-sions) and scarring corneal manifestation s within each major EB Subtype over time. Design Observational (cross-sectional and longitudinal). Methods Up to 16years of longitudinal follow-up was conducted on 3,280 consec-utively enrolled patients in the National EB Registry, an epidemiologic study funded by the Na tional Institutes of Health. Data were stratified by majo r EB type and subtype. Frequencies of occurrence were detemined for eight vari-ables (corneal erosions or blistering; corneal scarring; symblepharons; blepharitis; ectro pions; lacrimal duct obstructions; impaired vision; blind ess) by contingency ta-bles, and cumulative risks were generated by life table analysis technique. Results of the most common ocular manifestations were corneal erosio ns and blisters. Fre-quenci es mirrored relative severity of skin disease, with 74.10% of all patients with recessiv e dystrophic EB, Hallopeau-Siemens (RDEB-HS) and 47.50% of all pa- tients with junctional EB, Herlitz (JEB-H) experiencing at least one episode. Power frequencie s were noted for corneal scarring. Mitblepharons and ectrop ions were most com-monly seen in inversa RDEB and JEB-H, respectively. Blindness was reported in 6.47% of RD EB-HS patients. The cumulative risks of nonscarring and scarring corneal lesions in JEB-H age 5are 83.18% an d 27.08% and age age 25are 83.18% and 72.22% .With tim e, the cumu-lative risk of each in RDEB-HS approached that reported in JEB-H patients .Conclusion Ocular d isease activity, par-ticularly corneal, is common in some EB subtypes.Careful ophthalmologic examination should become an integral part of the management of all patients wit h inherited EB.