Rare Cause of Dysphagia

Abstract

Purpose: Introduction: Bullous diseases of the esophagus are rare causes of dysphagia.These diseases are often autoimmune and present with concomitant skin lesions. Patient Presentation: A 43-year-old woman presented with a skin rash that started one month previously. It spread from the right thigh to involve the whole body. Two weeks later the patient developed odynophagia to solids.She was started on valacyclovir with no improvement. Thus, she was admitted to the hospital for further management. At the time of admission she was on a tapering dose of cyclosporine secondary to failed kidney transplant. Examination revealed multiple flaccid vesicles containing clear fluid, with areas of crusting and hemorrhage. Systemic examination was unremarkable. A skin biopsy performed, showed bullous dermatitis with viral effects on keratinocytes: however cell culture and polymerase chain reaction for herpes simplex virus were negative. EGD showed a large area of ulceration and friability in the mid esophagus. A vesicular blistering lesion was seen in this area with the passage of the scope.Multiple biopsies were taken which revealed squamous mucosa with subepidermal blisters. A direct immunofluorescence test on the skin biopsy showed IgG/C3 linear deposits in the epidermal basement membrane. The patient's clinical picture was most consistent with Epidermolysis Bullosa Acquisita (EBA) and she was treated with steroids and cellcept with significant improvement in her symptoms. She is still being followed up to avoid long-term sequel, such as esophageal strictures. Discussion: EBA is a cutaneous subepidermal autoimmune blistering disorder that results from the effects of IgG autoantibodies directed against collagen VII. Patients present with tense blisters within non-inflamed skin.EBA may lead to mucosal damage and bullous formation in the esophagus from the trauma of food or reflux. These subsequently lead to formation of webs and strictures leading to dysphagia. During endoscopy there may be spontaneous bullous formation from manipulation. In severe cases there may be total mucosal desiccation of the esophagus. Biopsies however diagnostic, may lead to further trauma. Linear IgG/C3 deposits in the basement membrane at the dermal epidermal junction from both skin and esophagus help with the diagnosis. Circulating anti-basement membrane zone antibodies that bind to the floor of the blister on Direct Immunoelectron Microscopy are confirmatory. Linear IgA deposits on skin biopsy correlate with mucous membrane involvement. EBA has shown to be associated with inflammatory bowel disease, in particular crohn's disease. Treatment involves immunosuppresion with steroids, immunomodulators, intravenous immunoglobulins, dapsone or colchicine.