Melkersson-Rosenthal syndrome (MRS), also known as cheilitis granulomatosa or Miescher-Melkersson-Rosenthal syndrome, is a rare condition of unknown etiology characterized by the classic triad of:

• swelling of the face and lips

• facial nerve (CN VII) paralysis

• fissuring or furrowing of the tongue (lingua plicata)

Epidemiology

The syndrome is rare and typically manifests in young to middle-aged female adults, with a male to female sex ratio of 1:2 ⁵. Pediatric cases have been reported, but are exceptionally rare ⁵.

Associations

• Crohn disease (rare postulation) ²

• multiple sclerosis (rare postulation) ³

Clinical presentation

The classic triad, as aforementioned, consists of ^4,5:

• recurrent swelling of the face and lips

  • most common symptom

  • typically painless

  • typically unilateral

• facial nerve (CN VII) paralysis

  • lower motor neuron pattern

  • may be complete or partial, unilateral or bilateral (up to 40% of cases), and transient/recurrent or permanent (less common)

  • involvement of cranial nerves II, VIII, IX and XII have also been reported

• fissuring or furrowing of the tongue (lingua plicata)

  • may lead to loss of taste, loss of sensation, and tongue infections

  • permanent feature

Although classic, this triad is only seen in up to 25% of cases ⁴.

Pathology

The exact aetiopathogensis is not known, but is thought that immunogenic mechanisms play an important role ^4,5. In biopsy of an affected lip or part of the face, non-caseating granulomatous cheilitis is seen ⁵.

Radiographic features

Imaging findings are confined to confirming preseptal orbital edema ¹. Abnormalities of the facial nerve are not typically seen ¹.

Treatment and prognosis

Generally, management is typically with short courses of corticosteroids, although a number of other immunosuppressive agents have been trialed in the literature with varying benefit in small series or case reports ⁵. For facial swelling, intralesional triamcinolone acetonide injections may be helpful ⁵.

In patients with refractory disease, bony decompression of the facial nerve may help reduce the severity of facial palsy ^4,5. Furthermore, reconstructive surgical techniques may be employed for patients with permanent lip swelling ^4,5.

History and etymology

The condition was first described, albeit incompletely, by Melkersson in 1928 ⁴. The clinical description was further added to by Rosenthal in 1931 ⁴.

Differential diagnosis

• Bell palsy

• sarcoidosis (neurosarcoidosis)

• tuberculosis

• Lyme disease