Melkersson–Rosenthal syndrome

Autosomal dominant - en

Melkersson–Rosenthal syndrome (MRS) is a rare neurological disorder characterized by recurring episodes of facial swelling, particularly of the lips (known as cheilitis granulomatosa), recurrent facial paralysis, and the development of folds and furrows in the tongue (known as lingua plicata or fissured tongue). The syndrome is named after Swedish neurologist Ernst Melkersson and German otolaryngologist Curt Rosenthal, who first described the condition in the early 20th century.

Signs and Symptoms

The primary symptoms of Melkersson–Rosenthal syndrome include:

• Recurrent, non-painful swelling of the face, particularly the lips.
• Recurrent episodes of facial nerve paralysis, which may be unilateral or bilateral.
• Fissured tongue, characterized by deep grooves and furrows on the surface of the tongue.

Other symptoms may include:

• Swelling of other parts of the body, such as the eyelids.
• Headaches.
• Fever.
• Malaise.

Etiology

The exact cause of Melkersson–Rosenthal syndrome is unknown. However, it is believed to involve a combination of genetic and environmental factors. Some cases have been associated with family history, suggesting a possible genetic predisposition. Infections, allergies, and autoimmune disorders have also been proposed as potential triggers.

Diagnosis

Diagnosis of Melkersson–Rosenthal syndrome is primarily clinical, based on the characteristic triad of symptoms. Additional diagnostic tests may include:

• Biopsy of the affected tissue to identify granulomatous inflammation.
• Magnetic resonance imaging (MRI) or computed tomography (CT) scans to assess facial nerve involvement.
• Blood tests to rule out other conditions with similar presentations.

Treatment

There is no cure for Melkersson–Rosenthal syndrome, and treatment focuses on managing symptoms. Treatment options may include:

• Corticosteroids to reduce inflammation and swelling.
• Nonsteroidal anti-inflammatory drugs (NSAIDs) for pain and inflammation.
• Antibiotics if an underlying infection is suspected.
• Immunosuppressive drugs in severe cases.
• Physical therapy to improve facial muscle function.

Prognosis

The prognosis for individuals with Melkersson–Rosenthal syndrome varies. Some individuals may experience only a few episodes, while others may have recurrent or chronic symptoms. Early diagnosis and treatment can help manage symptoms and improve quality of life.

Related Pages

• Cheilitis granulomatosa
• Facial nerve
• Lingua plicata
• Neurological disorder
• Corticosteroids
• Autoimmune disorder