Pathology of Melkersson-Rosenthal Syndrome

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Dermpath-India

Dr Sampurna Roy MD

Melkersson-Rosenthal syndrome (MRS) is a rare systemic neuro-mucocutaneous granulomatous disease.

It is also known as cheilitis granulomatosa or Miescher-Melkersson-Rosenthal Syndrome.

Consists of a triad of:

1) Intermittent facial palsy.

2) Recurrent swelling of lips and/or orofacial oedema.

3) Plicated tongue.

(Note: In some cases the lesion may present in the orbit and periocular region with oedema of the eyelids.)

The etiology is unknown, however the following factors may be involved :

- Genetic and/or infectious factors.

- Allergic reactions to food or food additives

- Autoimmune Diseases

May be polyetiological disease with genetic or acquired predisposition associated with functional disturbance of autonomous nervous system leading to granulomatous reaction.

Microscopic features: A skin biopsy of swollen lip or facial tissues may show the following features.

- A marked upper dermal oedema

- Small epithelioid cell granulomas arranged in perivascular and perilymphatic location.

- Multinucleated Langhans-type giant cells.

- Perivascular mononuclear inflammatory infiltration.

- Areas of fibrosis

- Collections of small epithelioid cells may be occasionally observed within lymphatic spaces.

- No acid-fast bacteria, fungi or foreign bodies are detected.

Differential diagnosis:

Crohn's Disease; Tuberculosis ; Sarcoidosis ; Granulomatous Blepharitis ; Cheilitis ; Contact dermatitis.

The disease has a chronic progressive course and all patients should be followed up regularly.

Neurologists who observe a combination of facial edema and facial palsy in a patient should consider the diagnosis of MRS and proceed to a diagnostic skin biopsy and a trial of steroid treatment for their patient.