PARRY-ROMBERG SYNDROME

Named after the two first described, the English physician Caleb Hillier Parry General and the German neurologist Moritz Heinrich Romberg. The Parry-Romberg syndrome is a rare disease in which there is a slowly progressive, localized atrophy of the skin, subcutaneous fatty tissue, bones and muscles. Often start with a hyper-or hypopigmentation of the facial skin of the affected side or one-sided loss of hair.

Typically relates to the shrinkage of a face and begins in adolescence. In an unclear number of patients, there are also lesions and atrophy of the brain and are indeed on the same side as the Gesichtsatrophie, frequent occurrence of epileptic seizures, migraine facial pain on the affected side before concentrated. There is a relationship with known connective tissue diseases (scleroderma) and similarities of the Parry-Romberg syndrome with central lesions with Rasmussen's syndrome. An autoimmune cause is likely for the disease, but it also Lyme disease, trauma, genetic causes and trigeminal neuritis discussed as a cause.

The disease is on average after 7 to 9 years to a halt. Only a cosmetic surgery is reasonable. An effective causal treatment is not known. Experiments with penicillin are sometimes described as successful.Parry-Romberg syndrome is a rare disorder characterized by slowly progressive degeneration (atrophy) of the soft tissues of half of the face (hemifacial atrophy). Some individuals may experience distinctive changes of the eyes and hair, and neurological abnormalities including episodes of uncontrolled electrical disturbances in the brain (seizures) and episodes of severe pain in tissues supplied by the fifth cranial nerve (trigeminal nerve) including the mouth, the cheek, nose and / or other facial tissues (trigeminal neuralgia).

In rare cases, the disorder is apparent at birth. The majority of individuals with Parry-Romberg syndrome experience symptoms before the age of 20 years. In some rare cases, the atrophy may be bilateral. Affected areas may demonstrate shrinkage and atrophy of tissues beneath the skin (subcutaneous tissue), the layer of fat under the skin (subcutaneous fat) and underlying cartilage, muscle and bone. Many individuals also experience atrophy of half of the tongue and upper lip as well as abnormal exposure, delayed eruption, or wasting of the roots of certain teeth from the affected side. Symptoms of Parry-Romberg syndrome may begin at any age. Facial atrophy may cease abruptly or progress slowly and then become stationary. In other cases, the atrophy may progress indefinitely. The range and severity of symptoms and results linked may vary from case to case. In most cases, Parry-Romberg syndrome appears to occur randomly for unknown reasons (sporadically).