Melkersson syndrome is a rare neurological disorder characterized by a triad of symptoms: orofacial swelling, recurrent facial paralysis, and fissured tongue. It is named after the Swedish neurologist Ernst Melkersson, who first described the condition in 1928. The exact cause of Melkersson syndrome is unknown, although it is believed to have a genetic component and may involve an autoimmune response.
The most prominent feature of Melkersson syndrome is swelling of the face, typically affecting the lips, cheeks, and eyelids. This facial swelling, known as angioedema, may last for days or weeks and often recurs intermittently. Individuals with the syndrome may also experience recurrent episodes of facial nerve paralysis, causing drooping of one side of the face.
In addition to the orofacial manifestations, Melkersson syndrome is associated with a fissured or furrowed tongue, characterized by deep grooves or cracks on the surface of the tongue. Other less common symptoms include difficulty swallowing, loss of taste sensation, and ringing in the ears.
The diagnosis of Melkersson syndrome is primarily based on the clinical presentation of the characteristic triad of symptoms. However, further testing may be necessary to rule out other conditions with similar features, such as sarcoidosis or multiple sclerosis.
Treatment for Melkersson syndrome aims to manage the symptoms and may involve the use of medications such as corticosteroids to reduce facial swelling and relieve inflammation. In some cases, surgical interventions may be considered, including nerve decompression or facial reanimation procedures.
Overall, Melkersson syndrome is a rare disorder characterized by orofacial swelling, recurrent facial paralysis, and fissured tongue. While it can have a significant impact on an individual's quality of life, early diagnosis and appropriate treatment can help
