Melkersson Rosenthal Syndrome

Introduction:

This is a rare pathological entity with unidentified pathogenesis. The diagnostic criteria is also equivocal. This syndrome was first described by Melkersson and Rosenthal. There are three identified signs of this syndrome. They include:

Orofacial oedema

Facial nerve paralysis

Fissured tongue

Incidence:

The annual incidence of Melkersson Rosenthal syndrome ranges between 0.2 - 0.3 in 100,000 population per year.

Etiology:

The etiology largely remains indeterminate. Possible causes that has been attributed to this syndrome include:

Crohn's disease

Sarcoidosis

Herpes infection

Allergic reactions

Auto immune disorders

Diagnosis:

Dominant manifestation of this syndrome include:

1. Orofacial granuloma with oedema

2. Chelitis granulomatosa - This is the most frequently encountered type of manifestation

3. Edema of cheeks, tongue and eyelids have also been reported. These patients could experience recurrent short episodes of edema for many years, this becomes gradually persistent as the years go by. Edema of facial structures could mimic angioneurotic oedema, the difference being that it lasts longer and does not respond fo antihistamine administration.

4. Unilateral / bilateral facial palsy indistinguishable from that of Bell's palsy is also encountered. Facial nerve paralysis could become permanent after recurrent episodes of this syndrome. Other cranial nerve palsies have also been reported.

5. Fissured tongue - This is again an important manifestation of Melkersson Rosenthal syndrome. Fissured tongue is also known as Lingua plicata. This condition is seen in roughly about a third of these patients. Fissured tongue is classically described as the presence of fissures of atleast 2 mm deep and 15 mm long grooves. These fissures are found crossing the dorsum or margins of the tongue.

6. Acute anterior uveitis

7. GI symptoms

8. Trigeminal neuralgia

9. Psychotic episodes

10. Migraine

11. Long standing autoimmune disturbances

Histopathology:

Pathological studies of these lesions reveal the presence of non caseating granuloma, giant cells, lymphocyte infiltration and fibrosis.

Management:

There is no concensus regarding the management modality. Steroids form the main stay of the program.