Melkersson-Rosenthal syndrome with underlying sarcoidosis in a middle aged female patient

Abstract

Melkersson-Rosenthal syndrome (MRS) is a rare entity. It is often described as a triad of facial swelling, facial nerve palsy and a plicated tongue, but may be diagnosed when two of the above symptoms are seen. It is characterized by a granulomatous inflammation on histological examination and hence disorders such as Sarcoidosis, Inflammatory Bowel Disease and Tuberbulosis are implicated in the etiology. Response to treatment can be disappointing, with most patients having recurrent episodes over a chronic course. Here we present a case of a 45 year old woman who presented with gradual orofacial edema for the last 7 months which she first noted after her Covid vaccination. It was followed by the appearance of left sided facial nerve palsy. Upon investigation, we found radiological evidence of pulmonary sarcoidosis and raised serum Angiotensin Converting Enzyme levels. A biopsy from her lip showed granulomatous inflammation. We treated her with high dose systemic steroids, Mycophenolate Mofetil and Methotrexate. The combination of orofacial edema, facial nerve palsy and a fissured tongue (together called the Melkersson–Rosenthal syndrome) remains a rare presentation with less than 40 cases having been reported in literature. Investigating the patient may uncover an underlying diagnosis of a systemic granulomatous disorder such as Sarcoidosis, or other etiologies including infections and autoimmune conditions. There are no established guidelines towards treatment and the choice of therapy depends upon an individual case-by-case approach and the physician’s discretion.