Parryâ€“Romberg syndrome

The Parry - Romberg syndrome, also Progressive hemifacial atrophy, is a rare disease of unknown cause, in which there is typically a progressive unilateral atrophy ( hemiatrophy ) of the face. The disease was named after the two Erstbeschreibern Moritz Heinrich Romberg and Caleb Hillier Parry.

Epidemiology

The Parry - Romberg syndrome is a very rare disease in which the disease incidence is not known. It occurs primarily in young women ( Gynäkotropie ). At initial presentation, 75% of predominantly female patients under the age of 20 years

Cause

The cause of the disease is unclear. The cause is infection, especially with Borrelia, an autoimmune etiology, trauma and genetic factors discussed .. As a guide for an autoimmune etiology is the detection of antinuclear antibodies in more than half of the cases and the detection of rheumatoid factor in about one third of cases counted.

Disease

The disease is characterized by a single-sided progressive atrophy of some to all tissues of a face. Initial, a hypo-or hyperpigmentation of the affected facial skin exist. The atrophy of the skin and hyperpigmentation of the affected side is called " coup de saber ". Furthermore, adipose tissue, muscle, cartilage and bone may be involved. An involvement of the extremities has been reported.

It can migraine, trigeminal neuralgia, focal epilepsies occur. Have also been described depigmentation and hair loss on the affected side.

Diagnostics

The diagnosis is made considering the patient history and clinical examination findings. By imaging of the head ( magnetic resonance imaging ( MRI) or computed tomography ( CT) ) and by histopathological examination of the skin, the diagnosis can be based. In the Comuputertomografie hypodensities and calcifications may be detectable in participation of the brain. In magnetic resonance imaging can be detected an enhancement of the white matter and meninges.

Treatment and prognosis

The Parry - Romberg syndrome is a first progressive and self-limiting in the course of disease. On average, the disease activity lasts 7-9 years. A definitive curative therapy for the active phase of the disease is not known. However, there are numerous case reports in which various medications and other therapies have come with success to the application. These include

The use of corticosteroids and other immunosuppressive agents,
Treatment with antibiotics (eg, penicillin and ceftriaxone ), especially in the detection of Lyme disease,
And the use of UV-A radiation ( PUVA).

If no disease activity is detectable, that is when the disease is no longer progresses, reconstructive and plastic surgical procedures are possible. The project uses such as the pedicled fat -free plastic, with the help of the atrophic side of the face can be reconstructed. In addition, the use of autologous and lipoinjection of foreign body injection ( Hydroxyapatid granules ) have been described.