S2362 Paraneoplastic Mucous Membrane Pemphigoid: Not Your Typical Cause of Odynophagia

Abstract

Introduction: Paraneoplastic Mucous Membrane Pemphigoid (MMP) is a rare inflammatory autoimmune disorder that primarily affects the oral, ocular and aerodigestive mucous membranes and is associated with an underlying malignancy. IgG, IgA, and C3 autoantibodies target basal membrane zones causing subepithelial blisters with erosive lesions in the mucosa. It is rarely seen in the clinical setting, with an incidence of 2-10 cases in 100,000 individuals. High clinical suspicion is needed in order to diagnose Paraneoplastic MMP presenting as odynophagia and dysphagia. Case Description/Methods: A 88 year old female presented with a 5 month history of worsening odynophagia, dysphagia to solids and liquids and a fifty pound weight loss. Physical examination was remarkable for diffuse erosive lesions of the oral mucosa, tense bullae on the soft palate, and bilateral temporal symblepharon along with inflammation of upper and lower eyelids. Laboratory workup demonstrated elevated Cancer Antigen-125 (574.20 U/mL). Immunofluorescence study of biopsies taken from esophageal mucosa, right lateral tongue lesions and the left inferior conjunctiva were remarkable for IgG, IgM, IgA interkeratinocytic deposits at lower level of the epidermis and C3 linear deposits at the dermoepidermal junction consistent with Paraneoplastic Mucous Membrane Pemphigoid. Chest-abdominal-pelvic computed tomography scan demonstrated an irregular enhancing right adnexal mass, intra-abdominal lymphadenopathy, left hemiabdomen omental metastatic deposits and bilateral pulmonary nodules. Computed tomography guided core biopsy of a left omentum deposit was remarkable for primary ovarian metastatic high grade serous carcinoma. Due to refractory odynophagia and dysphagia, intravenous Solumedrol was initiated with subsequent improvement of symptoms. Patient was discharged with multidisciplinary team follow up for chemotherapy initiation and Rituxan induction. Discussion: Paraneoplastic MMP is an uncommon autoimmune mucocutaneous disease diagnosed when there is presence of stomatitis, histologic features of acantholysis or interface dermatitis, demonstration of anti-plakin autoantibodies on biopsy, with an underlying neoplasm. Less than 500 cases have been reported where clinical features, presentation, and autoantibodies profiles have varied widely between patients highlighting the challenge of diagnosis. Early recognition of Paraneoplastic MMP presenting as odynophagia is difficult due to the overlap of symptoms in more prevalent conditions.