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Dermpath-India

Pathology of Melkersson-Rosenthal Syndrome

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.

 

Further reading:

Melkersson Rosenthal syndrome: a histopathologic mystery and dermatologic challenge.

Granulomatous cheilitis of Miescher: the diagnostic proof for a Melkersson-Rosenthal syndrome.

Cheilitis Granulomatosa: A Review.

Melkersson-Rosenthal syndrome: a review of seven patients.

Melkersson-Rosenthal syndrome in a patient with psoriatic arthritis receiving etanercept.

Orofacial granulomatosis: A case report with review of literature.

The Melkersson-Rosenthal syndrome: a retrospective study of biopsied cases.

Cheilitis granulomatosa (Miescher granulomatous macrocheilitis) with trisomy 21.

Melkerssons-Rosenthal syndrome: a case report and review of the literature.

Orofacial granulomatosis presenting as persistent lip swelling: review of 6 new cases.

The immunohistochemical and electron microscopical study of pathological classification of Melkersson-Rosenthal syndrome .

 

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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