Corneal Ulcer and Epidermolysis Bullous Acquisita, an Unsual Presentation of Inflammatory Bowel Disease (IBD)

Abstract

Inflammatory bowel disease (IBD) is a systemic inflammatory disorder that has specific predilection for gastrointestinal tract but can affect multiple organs including eyes, that can present as keratitis, episcleritis or uveitis causing significant visual morbidity. These ocular manifestations may present before the usual intestinal signs and symptoms. We report an extremely rare case of corneal ulceration with epidermolysis bullous acquisita (EBA) in a patient with no gastrointestinal symptoms. A 37-year-old female presented to the emergency department with severe sharp left eye pain and photophobia. The eye discomfort started five days ago and gradually worsened. Patient also reported an ulcerative lesion on the dorsum of right hand and both legs that started as blisters and gradually developed into ulcers over the past 10 days. She had low-grade fevers at times, but denied weight loss or any history of sexually transmitted diseases including syphilis and herpes. Ophthalmological exam revealed left eye's corneal ulceration. Since there was a concern for herpes ophthalmicus and bacterial infection, patents was started on vancomycin and tobramycin along with intravenous acyclovir but were discontinued later as corneal cultures did not grow any organisms. Skin lesions on hand and legs were compatible with a presentation of epidermolysis bullous acquisita (figure 1 and 2), confirmed by skin biopsy. Blood work-up revealed iron deficiency anemia, hence she underwent a colonoscopy that showed focal ulcerations with adjacent normal mucosa, cobblestoning, thickened wall and long fissures. Biopsy confirmed Crohn's disease. Crohn's disease is a chronic inflammatory bowel disease characterized by transmural inflammation of the gastrointestinal tract, and 4-8% patients have been reported to have ocular manifestations. However corneal ulcerations are far less common than conjunctivitis, episcleritis and uveitis. EBA is a chronic autoimmune skin disease and can involve mucous membranes. Antibodies target type VII collagen; fibrils connecting basement membrane to dermis, and presents as blisters that can develop into dense scars on extensor surfaces or trauma prone areas along with mild mucosal lesions. Corneal ulceration and epidermolysis bullous acquisita in an undiagnosed IBD have rarely been reported and hence considering IBD as a differential in such presentations leads to early diagnosis and treatment preventing visual morbidity.Figure: Hand Ulcer.Figure: Leg Ulcers.