The defining components of Melkersson-Rosenthal syndrome (MRS) are lingua plicata, facial nerve palsy that may be unilateral or bilateral, partial or complete [1] [2] and orofacial edema or granulomatous cheilitis [3] [4]. The disease has a recurrent character, with intermittent relapses and asymptomatic periods. Episodes tend to become worse and more prolonged as the disease progresses. It is sometimes associated with other conditions, such as sarcoidosis, tuberculosis, leprosy, histoplasmosis, foreign body reactions, granulomatosis with polyangiitis, mixed connective tissue disease, Bell's palsy and Crohn's disease, in which case patients also exhibit symptoms of the underlying pathology. Some patients may have only one or two of the three components of the disease. Melkersson-Rosenthal syndrome can be a manifestation of Crohn's disease, sarcoidosis, and orofacial granulomatosis, so these entities should be ruled out first when a patient presents.

Granulomatous cheilitis manifests as diffuse or nodular, soft or firm swelling of the lips and/or surrounding tissues. The lips may appear cracked and discolored and eventually become painful. If the syndrome appears as part of Crohn's disease, affected mucosa takes the typical "cobblestone" appearance and may ulcerate [5]. Cheilitis can be asymmetric or unilateral and can also become permanent. The edema may involve neighboring structures, such as the eyelids, palate, gingiva, larynx and pharynx. The affected lip becomes painful, firm, changes color over time and becomes fissured. The initial symptoms usually resolve while subsequent episodes may be more severe, last longer or fail to disappear. The patient may also exhibit fever, headache, and visual disturbances. Additional facultative traits include diminished salivary gland secretion, decreased gustatory sensation, mask-like facies, thick lower lip vermilion, furrowed tongue, abnormal temperature regulation and other autonomic nervous system abnormalities, lymphadenopathy, and nystagmus. The olfactory, auditory, glossopharyngeal and hypoglossal nerves may also be affected.

The central nervous system may be involved in some cases, causing multiple sclerosis-like symptoms or miscellaneous psychiatric symptoms and autonomic disturbances.

The diagnosis of Melkersson-Rosenthal syndrome is based on clinical findings. If needed, skin biopsies may be performed and they reveal different features, depending on the stage of the disease. In early stages, lymphedema and perivascular lymphocytic infiltration are found, while later in the evolution of the disease non-caseating granulomas around vessels have been described [6]. However, it is important to rule out Crohn's disease and sarcoidosis, and that is achieved by performing a biopsy of the lip or surrounding tissues. If the result is unsatisfactory, a colonoscopy is indicated to exclude Crohn's disease [7], and gallium or positron emission tomography scans are recommended to rule out sarcoidosis and tuberculosis. A serum angiotensin-converting enzyme test can also be used to detect sarcoidosis. Granulomatous cheilitis may occur as a result of metal or food additives sensitivity, and patch tests with these antigens are useful to diagnose it [8].

There is no cure for MRS, but treatment focuses on managing symptoms:

• Corticosteroids: These anti-inflammatory drugs are often used to reduce swelling and inflammation.
• Immunosuppressants: In some cases, drugs that suppress the immune system may be prescribed.
• Physical Therapy: For facial palsy, exercises may help improve muscle strength and coordination.
• Surgery: Rarely, surgical intervention may be considered for persistent swelling or cosmetic concerns.

Treatment is tailored to the individual, and a multidisciplinary approach may be beneficial.

The prognosis for MRS varies. Some patients experience mild symptoms with long periods of remission, while others may have more frequent and severe episodes. The condition is chronic, and while it can be managed, it often requires ongoing treatment and monitoring.

The exact cause of MRS is unknown. It is believed to involve a combination of genetic, environmental, and immunological factors. Some cases have been linked to family history, suggesting a possible genetic predisposition.

MRS is a rare condition, with no precise data on its prevalence. It can occur at any age but is most commonly diagnosed in young adults. There is no clear gender or ethnic predilection, although some studies suggest a slight female predominance.

The pathophysiology of MRS is not fully understood. It is thought to involve an abnormal immune response leading to granulomatous inflammation, particularly in the facial tissues. This inflammation causes the characteristic swelling and can affect nerve function, leading to facial palsy.

There are no known preventive measures for MRS due to its unclear etiology. Managing triggers, such as stress or infections, may help reduce the frequency of episodes in some patients.

Melkersson-Rosenthal Syndrome is a rare, chronic condition characterized by facial swelling, facial palsy, and a fissured tongue. Diagnosis is based on clinical presentation and exclusion of other conditions. While there is no cure, symptoms can be managed with medication and supportive therapies. The cause remains unknown, and the condition can vary significantly in its course and severity.

If you have been diagnosed with Melkersson-Rosenthal Syndrome, it's important to work closely with your healthcare provider to manage your symptoms. Treatment may involve medications to reduce inflammation and improve facial muscle function. Regular follow-up appointments can help monitor your condition and adjust treatment as needed. Understanding your condition and recognizing potential triggers can also aid in managing symptoms effectively.

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