PURPOSE: To report clinical and laboratory findings of bilateral corneal involvement in a patient with epidermolysis bullosa acquisita. METHODS: A 25-year-old man with a history of progressive and painless loss of vision in both eyes presented to our service with bilateral corneal involvement: peripheral corneal perforation in one eye and advanced corneal thinning in the other eye. There was concomitant dermatologic bullous disease. Clinical and laboratory exams were analyzed. RESULTS: The patient was diagnosed as having epidermolysis bullosa acquisita. Therapeutic corneal patch graft and conjunctival resection with cryotherapy were done, with satisfactory results. CONCLUSION: Bilateral corneal involvement in epidermolysis bullosa acquisita is described. To the best of our knowledge, this is the first description of such a case. Surgical management of the ocular findings associated with systemic therapy with colchicine seems to be a good therapeutic option in the management of this defying disease.
PURPOSE: To report clinical and laboratory findings of bilateral corneal involvement in a patient with epidermolysis bullosa acquisita. METHODS: A 25-year-old man with a history of progressive and painless loss of vision in both eyes presented to our service with bilateral corneal involvement: peripheral corneal perforation in one eye and advanced corneal thinning in the other eye. There was concomitant dermatologic bullous disease. Clinical and laboratory exams were analyzed. RESULTS: The patient was diagnosed as having epidermolysis bullosa acquisita. Therapeutic corneal patch graft and conjunctival resection with cryotherapy were done, with satisfactory results. CONCLUSION:Bilateral corneal involvement in epidermolysis bullosa acquisita is described. To the best of our knowledge, this is the first description of such a case. Surgical management of the ocular findings associated with systemic therapy with colchicine seems to be a good therapeutic option in the management of this defying disease.
Authors: Ruchi Shah; Cynthia Amador; Kati Tormanen; Sean Ghiam; Mehrnoosh Saghizadeh; Vaithi Arumugaswami; Ashok Kumar; Andrei A Kramerov; Alexander V Ljubimov Journal: Exp Eye Res Date: 2021-01-21 Impact factor: 3.467
Authors: Brendon W H Lee; Jeremy C K Tan; Melissa Radjenovic; Minas T Coroneo; Dedee F Murrell Journal: Orphanet J Rare Dis Date: 2018-05-22 Impact factor: 4.123