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Osteo‐odonto‐keratoprosthesis restores vision in ocular mucous membrane pemphigoid

we report the case of a 51-year old Caucasian man who presented himself at our hospital with ocular involvement of mucous membrane pemphigoid (MMP). Both eyes were affected by intense scarring, symblephara and erythema of the conjunctiva. His vision was reduced to 0.1–0.15 in the right eye, while in the left eye he was nearly blind, with only blurry vision remaining. He had been diagnosed with ocular mucous membrane pemphigoid (ocMMP) ten years before and had received a recurrent high-dose prednisolone treatment, a concomitant therapy with dapsone followed by cyclophosphamide. Due to the additional involvement and rapid deterioration of the skin of the face, scalp and arms, the systemic therapy was replaced with mycophenolate sodium and further supplemented by rituximab infusions [1, 2]. As a result, the skin lesions healed completely, and the eye inflammation regressed. He remained in ophthalmologic care for seven years, with varying topical eye treatment (Figure 1). The diagnosis of MMP was based on histopathological visualization of subepidermal blistering and positive direct immunofluorescence showing linear deposits of IgG and C3 along the basement membrane zone [1]. Immunoblotting detected circulating IgG4 autoantibodies against laminin 332 that can be found in approximately 20 % of all MMP patients [3–5]. In particular, the oral mucosa (85 %) and eyes (65 %) are affected by the disease [6]. Patients with anti-laminin 332 ocMMP have an increased risk for associated malignancies. About 30 % of the cases are diagnosed with cancer, particularly of visceral organs such as the stomach and colon [4, 7, 8]. Our patient underwent repeated tumor screening tests, all of which returned negative. Initially, the patient was started on an intravenous immunoglobulin (IVIG) therapy to improve a recurrent inflammation of the conjunctiva and to restore his vision. In addition, a new regime of mycophenolate mofetil and subsequent cyclophosphamide was given. Despite the highly potent treatment, no improvement in vision could be achieved (Figure 1). At this point, the patient was only able to identify hand movements (Figure 2). Once blindness manifests, few therapeutic options remain. Previous amniotic membrane transplantations on the left and right eye had reduced the inflammation but did not improve the vision. As corneal transplantation frequently fails, visual rehabilitation in MMP associated with severe dry eye can only be achieved with the help of keratoprostheses, such as the osteo-odonto-keratoprosthesis (OOKP) or the tibial bone keratoprosthesis (TKpro) [9–13]. Long term analysis revealed better results for the OOKP, though the TKpro presents a useful alternative, if no suitable tooth is available [9, 10]. Our patient was therefore referred to the Department of Ophthalmology at the Ortenau Klinikum at Offenburg, which is one of two centers in Germany offering OOKP DOI: 10.1111/ddg.14259 Osteo-odonto-keratoprosthesis restores vision in ocular mucous membrane pemphigoid Clinical Letter