Successful treatment of mucous membrane pemphigoid with infliximab.

Our patient was diagnosed with cicatricial pemphigoid (CP) in 2001. She demonstrated classic clinical findings and underwent biopsies that supported this diagnosis. She unfortunately experienced severe CP involving both of her eyes, oral mucosa, pharyngeal mucosa, and esophagus. Her left eye was seriously affected early in the course of her disease. She experienced significant scarring of the cornea of her left eye as well as of both its upper and lower eyelids, resulting in symblepharon and complete blindness of this eye. She has also endured considerable disease in her right eye and has undergone multiple corneal transplants of this eye (in March 2001, September 2004, January 2005, and April 2005). The most recent corneal transplant in April 2005 was performed emergently for a ruptured corneal ulcer. Our dermatology team was consulted during the patient’s hospitalization for this event. At our initial encounter with the patient, she had complete blindness of her left eye and had recently undergone emergent corneal transplant surgery on her right eye. The right eye was sutured closed and bandaged. On examination, we observed multiple erosions of her soft palate and buccal mucosa (Figure 1). She reported difficulty eating for the past several years. This difficulty had progressed to the point where she could tolerate only soft, bland foods, and her pain significantly affected her oral intake and nutrition. Our patient had previously received multiple therapies to treat her CP, including prednisone, cyclophosphamide, and dapsone. She had received one 5-day course of intravenous immunoglobulin; however, she had no response to this treatment and was also felt to be a poor candidate for it given her risk of congestive heart failure. Her comorbid medical problems included diabetes mellitus, coronary artery disease requiring stent placement, and high blood pressure. None of the treatments for her CP resulted in substantial improvement, and her disease continued to progress.