What Is Melkersson-Rosenthal Syndrome?
MRS or Melkersson-Rosenthal syndrome is a disease or a syndrome characterized by orofacial granulomatosis and facial nerve paralysis. It is characterized by a triad of clinical features that are mainly,
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Facial Swelling - Facial swelling may also include lip swelling predominantly.
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Facial Paralysis - Facial paralysis may be peripherally present as drooping of the face unilaterally or to one side and with partial or complete inability to move the facial parts on the affected side of the face. It may also be recurring in nature.
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Fissured Tongue - Fissured tongue is commonly detected in the oral cavity by the dental surgeon or the patient themselves on routine observation (that may be attributed due to numerous underlying causes) but is important in the diagnosis of MRS when it presents alongside facial paralysis and swelling.
What Are the Clinical Features of Melkersson-Rosenthal Syndrome?
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Gender - Though it is an extremely rare disease, MRS predominantly affects women.
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Race - The prevalence of it in any one particular race is unknown; the racial occurrence of MRS is usually found to be even.
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Commonly Observed - Amongst the classic triad of features that is categorized in MRS, the most commonly observed feature is that of facial swelling only in 80 to 90 % of MRS patients, and the complete triad inclusive of facial paralysis accompanied by lip swelling and fissured tongue presents only in 25 to 30 % cases with MRS.
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Pathologic Examination - Microscopically, the cells are affected when the edematous tissue of the face is sent for pathologic examination, and they clearly reveal the presence of giant cells or Langerhans cells and infiltration by inflammatory cells or lymphocytes and epithelial cell granulomas that are non-classified. Fibrosis can be seen around the blood vessels, and mononuclear cells can also be observed under microscopy.
Triad of Clinical Features:
1. Facial Swelling - It is the most observed feature in MRS. The patient may feel the facial swelling is generally not so painful and ipsilateral (on one affected side) only. The most common site of inflammation or facial edema is the upper lip, followed by less frequent prevalence on other parts of the face like palate, tongue, gingiva, pharynx region, larynx region, periorbital region, or even the cheeks.
2. Facial Paralysis - This is observed typically in 45 to 90 % of patients with a bilateral involvement less often than unilateral involvement (incapability to move the facial muscles) is most reported. Even in some cases of post-treatment modalities by the physician, there is the likelihood of a paralytic feature on one side of the face permanently.
3. Fissured Tongue - Most patients suffering from MRS are present with both fissured tongue and geographic tongue (erythema migrans) in simultaneous conjunction. The difference between fissure tongue and geographic tongue though they may appear similar clinically, is that in geographic tongue, unlike grooving or fissuring or burrowing in the dorsum of the tongue, areas of depapillation and atrophy present on the tongue surface dorsally that appears as a map like projection with well-demarcated margin or border areas.
Clinical Facts:
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The important clinical fact that remains in both the conditions, the geographic or fissured tongue, is present with different morphologies, still are of benign origin, and found under routine dental examination.
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They are not cancerous or malignant, and they do not have the potential to be precancerous either.
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Malodor and discoloration are associated with a fissured tongue seen in MRS due to food deposits and bacterial accumulation in the cracks or the grooves of the tongue. The burning sensation may be associated sometimes with fissured tongue.
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The fissured tongue may also be associated with other underlying conditions of the body or systemic diseases, but when accompanied by facial swelling can definitely prove a classic diagnosis in detecting MRS.
Cause:
Psoriasis and tobacco consumption (nicotine addictions) can also be a potent aggravator as well as the cause of deep fissuring in such cases. The cause or pathophysiology of fissured tongue and geographic tongue may be genetic in origin also.
Fissured and Geographic Tongue:
When fissuring is also associated with geographic tongue, concentricity is observed as a pattern on the tongue or rather as a central groove that has smaller grooves radiating outwards from the central point of the tongue. The surface of the tongue may also appear crisscrossed in some cases, with the grooves having a penetrable depth of around 2 to 6 mm with a central midline fissure.
Management:
Though there is no curative treatment for tongue fissuring other than proper oral hygiene and maintenance, tongue brushing regularly every day can help prevent the accumulation of deposits and food within the fissures or grooves.
What Is the Differential Diagnosis of Melkersson-Rosenthal Syndrome?
The differential diagnosis by the oral maxillofacial surgeon while examination of Melkersson-Rosenthal syndrome includes potential conditions that would cause facial edema or swelling include:
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Herpes labialis.
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Crohn's disease.
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Sarcoidosis.
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Lymphangiomas.
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Hemangiomas.
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Hereditary angioedema.
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Erysipelas that are recurring.
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Superior vena cava syndrome.
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Eosinophilia.
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Submucosal neoplasms.
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Hypothyroidism.
Other conditions that involve peripheral facial paralysis for differential diagnosis include: ·
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Bell's palsy.
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Gillian Barre syndrome.
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Myasthenia gravis.
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Infectious mononucleosis.
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Leukemia.
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Herpes zoster infection.
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Otitis media.
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Multiple sclerosis.
If the patient has no previous or family history of allergic reactions, inflammations, or genetic tracing that shows any disease as MRS, then the differential diagnosis should be investigated thoroughly before concluding the diagnosis clinically by considering the classic triad symptoms for MRS.
What Is the Treatment for Melkersson-Rosenthal Syndrome?
Treatment for this rare but acute paralytic condition definitely depends on examination by the physician and dental surgeon after the clinical diagnosis of the symptomatic triad. Serologic evaluation can help in the detection of high C reactive proteins that may be useful in diagnosis. There is no single treatment strategy for curing patients suffering from MRS because accessory complications linked with other diseases or disorders like migraine, trigeminal neuralgia, psychosis, or ulcerative colitis is often a manifestation post a patient suffering from long term MRS.
Hence to prevent further complications and to cure the recurrent bouts of facial nerve paralysis, the physician will recommend a steroidal treatment for a selected duration, and with the help of anti-inflammatory and antibiotic drugs, recovery is possible though not completely. In patients with permanent deformation resulting from paralysis, the maxillofacial surgeon or plastic or cosmetic surgeons can be referred by the physician for facial reconstruction. But these reconstructive procedures can be performed when the patient has completely recovered from paralytic attacks.
Conclusion
Though MRS or Melkersson-Rosenthal syndrome is rare, it has an acute psychosomatic impact on the patient, and hence diagnosis and treatment planning go hand in hand to help the patient recover at the earliest possibility. Likewise, the physician and maxillofacial surgeon also need to coordinate in the full-fledged treatment of a patient affected by MRS.
