Ocular Involvement in Epidermolysis Bullosa Acquisita

IgA‐epidermolysis bullosa acquisita in a child resulting in blindness

“…These patients suffered from conjunctivitis, symblepharon formation, keratitis, subepithelial vesiculation, perforation and opacification of the cornea. 19,[21][22][23] In one of the two cases of IgA-EBA, ocular involvement was severe, resulting in total blindness, as in our patient. 12 Severe ocular lesions may also be observed in the recessive form of dystrophic epidermolysis bullosa which is associated ultrastructurally with absence of anchoring fibrils in the conjunctiva and skin, and associated with mutations in type VII collagen.…”

IgA‐epidermolysis bullosa acquisita in a child resulting in blindness

“…These patients suffered from conjunctivitis, symblepharon formation, keratitis, subepithelial vesiculation, perforation and opacification of the cornea. 19,[21][22][23] In one of the two cases of IgA-EBA, ocular involvement was severe, resulting in total blindness, as in our patient. 12 Severe ocular lesions may also be observed in the recessive form of dystrophic epidermolysis bullosa which is associated ultrastructurally with absence of anchoring fibrils in the conjunctiva and skin, and associated with mutations in type VII collagen.…”

Ocular involvement in IgA-epidermolysis bullosa acquisita

“…It is known that EBA may exhibit ocular involvement. 20–22 The presence of IgG in the conjunctival mucosa has recently been demonstrated by direct IEM in a patient with EBA. 19 We detected IgA in the conjunctiva of patient 1 and, to our knowledge for the first time in the literature, in the cornea, by DIF.…”

A review of scoring systems for ocular involvement in chronic cutaneous bullous diseases

“…Although ocular involvement has been documented in the literature, the incidence or prevalence has not been recorded [ 77 – 81 ]. Reported manifestations include keratitis, corneal vesiculation, and frequent symblepharon formation – however, vision loss is rare [ 40 , 80 ]. Ulcerative keratitis in the absence of scarring has also been noted, suggesting corneal inflammation could be a direct manifestation of EBA [ 57 ].…”

IgA‐epidermolysis bullosa acquisita in a child resulting in blindness

“…These patients suffered from conjunctivitis, symblepharon formation, keratitis, subepithelial vesiculation, perforation and opacification of the cornea. 19,[21][22][23] In one of the two cases of IgA-EBA, ocular involvement was severe, resulting in total blindness, as in our patient. 12 Severe ocular lesions may also be observed in the recessive form of dystrophic epidermolysis bullosa which is associated ultrastructurally with absence of anchoring fibrils in the conjunctiva and skin, and associated with mutations in type VII collagen.…”

Ocular involvement in IgA-epidermolysis bullosa acquisita

“…It is known that EBA may exhibit ocular involvement. 20–22 The presence of IgG in the conjunctival mucosa has recently been demonstrated by direct IEM in a patient with EBA. 19 We detected IgA in the conjunctiva of patient 1 and, to our knowledge for the first time in the literature, in the cornea, by DIF.…”

A review of scoring systems for ocular involvement in chronic cutaneous bullous diseases

“…Although ocular involvement has been documented in the literature, the incidence or prevalence has not been recorded [ 77 – 81 ]. Reported manifestations include keratitis, corneal vesiculation, and frequent symblepharon formation – however, vision loss is rare [ 40 , 80 ]. Ulcerative keratitis in the absence of scarring has also been noted, suggesting corneal inflammation could be a direct manifestation of EBA [ 57 ].…”

IgA‐epidermolysis bullosa acquisita in a child resulting in blindness

“…These patients suffered from conjunctivitis, symblepharon formation, keratitis, subepithelial vesiculation, perforation and opacification of the cornea. 19,[21][22][23] In one of the two cases of IgA-EBA, ocular involvement was severe, resulting in total blindness, as in our patient. 12 Severe ocular lesions may also be observed in the recessive form of dystrophic epidermolysis bullosa which is associated ultrastructurally with absence of anchoring fibrils in the conjunctiva and skin, and associated with mutations in type VII collagen.…”

Ocular involvement in IgA-epidermolysis bullosa acquisita

“…It is known that EBA may exhibit ocular involvement. 20–22 The presence of IgG in the conjunctival mucosa has recently been demonstrated by direct IEM in a patient with EBA. 19 We detected IgA in the conjunctiva of patient 1 and, to our knowledge for the first time in the literature, in the cornea, by DIF.…”

A review of scoring systems for ocular involvement in chronic cutaneous bullous diseases

“…Although ocular involvement has been documented in the literature, the incidence or prevalence has not been recorded [ 77 – 81 ]. Reported manifestations include keratitis, corneal vesiculation, and frequent symblepharon formation – however, vision loss is rare [ 40 , 80 ]. Ulcerative keratitis in the absence of scarring has also been noted, suggesting corneal inflammation could be a direct manifestation of EBA [ 57 ].…”