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Corneal Ulcer in a Patient with Crohn´s Disease Associated with Epidermolysis Bullosa Acquisita

Moch B. M., Spraul C. W., Lang G. E.,
Universität Ulm, Augenklinik und Poliklinik (Ulm)

Purpose: Crohn´s disease is a chronic inflammatory bowel disease, leading to ocular manifestations in 4-8% of patients. We report a patient with Crohn´s disease, who developed in association with epidermolysis bullosa acquisita a progressive corneal ulcer.
Method: A 26-year-old patient with a 3-year history of Crohn´s disease presented with a peripheral corneal ulcer of the right eye. The anterior chamber was deep and quiet. Fundus examination was normal. Corneal smears displayed no bacterial growth. Visual acuity of the right eye was 20/25. Examination of the left eye was normal. Furthermore, he had developed cutaneous blisters on the arms and legs.
Results: Despite treatment with topical antibiotics and systemic immunosuppressive therapy with tacrolimus, azathioprine and methylprednisolone the corneal manifestation deteriorated. Therefore, an amniotic membrane was transplanted for treatment of the corneal ulcer, however, this was followed by melting of the amniotic membrane within a few days. Additional therapeutic procedures including further amniotic membrane transplantations and autologous conjunctival grafts have been performed. This was regularly unsuccessful. The corneal ulcer increased in size and extended into the sclera. Therefore a tectonic sclerokeratoplasty was necessary. Histologic examination of the excised corneoscleral tissue revealed an extensive stromal thinning associated with inflammatory infiltration of the cornea and sclera.
Conclusions: Corneal complications in patients with Crohn´s disease are very rare in contrast to conjunvtivitis, episcleritis und uveitis. To the best of our knowledge, this is the first description of a patient with Crohn´s disease and epidermolysis bullosa acquisita developing progressive corneal ulcer. How much a supposed autoimmune mechanism in the cornea is related to Crohn´s disease or epidermolysis bullosa acquisita, is not known.