Melkersson Rosenthal syndrome

By

Dr. T. Balasubramanian M.S. D.L.O.

Introduction:

Melkersson – Rosenthal syndrome is a rare, non-caseating granulomatous disease which is characterised by a triad which includes facial paralysis, orofacial oedema and lingua plicata (scrotal tongue, fissured tongue, or furrowed tongue). It should be stressed that these triad of symptoms is not frequently seen in its complete form. The diagnosis of this condition is rather difficult because the classic triad of symptoms are seen only in 10% of patients with Melkersson – Rosenthal syndrome.

Etiology:

Etiology of this disease remains largely unknown however the following factors have been hypothetically implicated:

1. Infection

2. Genetic causes

3. Allergy

4. Benign lymphogranulomatosis

Histopathology:

Characteristic histopathologic features of this condition include:

1. Lymphoedema

2. Non caseating epitheloid cell granulomas

3. Presence of multinulceated Langhan's type giant cells

4. Presence of perivascular mononuclear inflammatory cell infiltration

5. Presence of perivascular fibrosis

Clinical features:

Patients with Melkersson Rosenthal syndrome may demonstrate the classic triad simultaneously or at different times. It should be stressed that in a patient with orofacial oedema the presence of one of the features given below is sufficient to make a diagnosis of Melkersson Rosenthal syndrome.

1. Idiopathic facial palsy

2. Lingua plicatica

Facial oedema:

This happens to be one of the dominant signs of Melkersson Rosenthal syndrome. The features of facial oedema in these patients include:

1. Acute oedema

2. Non pitting odema

3. Painless

4. Commonly affecting upper lip

5. Facial oedema may last from hours to weeks and may also recur

This facial oedema should be differentiated from angioneurotic oedema by its persistent nature, and non responsiveness to antihistamins and. Oedema is caused by fibrosis around blood vessels causing extravasation of fluid from them. The fibrosis also prevents reabsorption of extravasated fluid.

Facial paralysis:

In these patients facial paralysis may occur months to years before or after the onset of facial oedema. Facial palsy is commonly LMN type, unilateral / bilateral, partial or complete. 90% of these patients had recovery of facial nerve function.

Lingual Plicata:

Otherwise known as scrotal tongue / fissured tongue is commonly considered to be congenital developmental malformation.

Radiological evaluation which includes CT / MRI/Chest x-rays are non contributary in these patients. Histopathological features are the only available confirmatory diagnostic evaluation.

Other features associated with Melkersson Rosenthal syndrome:

1. Trigeminal neuralgia

2. Paresthesias

3. Ocular palsies

4. Blepharospasm

5. Epiphora

6. Keratitis

7. Psychotic episodes

8. Migraine

Treatment: Purely symptomatic.

Systemic / intralesional steroid therapy.

Drugs that have been tried with varying degrees of success include:

1. Sulfasalazine

2. Metronidazole

3. Clofazimine

4. Hydroxychloroquine