C40-10 Melkersson-Rosenthal Syndrome: A Rare Complication of Multisystem Sarcoidosis

Abstract Introduction Melkersson-Rosenthal Syndrome (MRS) is a rare complication of granulomatous diseases, most often associated with inflammatory bowel disease and sarcoidosis. It occurs more frequently in women, and consists of a triad of facial nerve palsy, fissured or plicated tongue, and orofacial edema. Case A 52-year-old woman with a history of neuro-ophthalmic sarcoidosis presented with swelling of her upper lip over the last several days. The patient’s initial symptoms began 18 years prior to her presentation, when she developed blurred vision and unilateral Bell’s palsy. Physical examination was significant for bilateral parotid and lacrimal gland enlargement as well as erythema nodosum. Ophthalmic examination showed anterior uveitis. Brain MRI demonstrated enhancement of the V2 branch of the trigeminal nerve. Chest CT identified bilateral hilar lymphadenopathy. Transbronchial biopsies revealed non-caseating granulomatous inflammation confirming a diagnosis of neuro-opthalmic sarcoidosis. She was treated with oral prednisone and intravenous infliximab, with resolution of her facial nerve palsy, erythema nodosum, and brain MRI findings. The prednisone dose was then slowly tapered off. Eight months before her presentation, the infliximab dosing interval was gradually increased, and the medication was discontinued 2 months prior to presentation. She then developed progressive swelling of her upper lip. She did not have pruritus, had not been started on any new medications, had not eaten unusual foods, and was not on ACE inhibitors. Due to her known diagnosis of neuro-sarcoidosis and prior history of facial nerve palsy, the new onset of facial swelling led to a diagnosis of MRS. She was started on prednisone and methotrexate, and lip swelling gradually resolved after several weeks. Discussion Components of the MRS triad may predate others by years, and only 25% of patients present with the complete triad. The diagnosis can be made based on the presence of any two components in a patient with risk factors. It is imperative to consider MRS in the differential diagnosis for patients at risk, like our patient with neuro-sarcoidosis, regardless of the timing of symptom onset. Anecdotal evidence supports treatment with corticosteroids and immunomodulatory agents, but relapses are common. This abstract is funded by: None