Parry -Romberg syndrome is a disease with a very low prevalence in the population. As the disease progresses, sufferers develop progressive atrophy, usually affecting one half of the face. The atrophy develops continuously over a long period of time.
What is Parry-Romberg Syndrome?
Parry -Romberg syndrome is also known in medicine as hemifacial atrophy or progressive facial hemiatrophy. It is a very rare disease whose exact triggers have not yet been adequately researched. In the context of Parry-Romberg syndrome, those affected suffer from atrophy or hemiatrophy, which shows up on half of the face. See ezhoushan for What does RBD Stand for.
The name of the disease derives from the two doctors who first scientifically described Parry-Romberg syndrome. These are Caleb Parry and Moritz Romberg. The exact frequency of Parry-Romberg syndrome has not yet been researched.
However, previous observations of patients show that the Parry-Romberg syndrome affects female patients in the majority of cases. These are often relatively young. About three quarters of the cases of the disease are found in women who have not yet completed their second decade of life.
There are currently no medical research studies that address the exact causes of Parry-Romberg syndrome. The lack of knowledge regarding the pathogenesis of the disease is also due to the rarity of the disease. It is assumed, however, that the unilateral atrophy of the face is due to nerve damage.
This lesion results in reduced blood supply to each half of the face. As a result, a lack of oxygen and nutrients leads to atrophy or hemiatrophy of the tissue in the face. In addition, other potential causes of Parry-Romberg syndrome are under discussion.
For example, some researchers assume that certain bacteria, such as Borrelia, are involved in the development of Parry-Romberg syndrome. Also, autoimmune diseases are considered as causes of the disease. In many people suffering from Parry-Romberg syndrome, the atrophy of one side of the face stops by itself after a certain time.
In addition, the possibility that genetic characteristics or traumatic events are responsible for the development of Parry-Romberg syndrome is discussed. The presence of so-called antinuclear antibodies, which are present in more than 50 percent of patients, suggests that the body’s own defense system is involved. In addition, rheumatism markers are identified in about 30 percent of the cases of the disease.
Symptoms, Ailments & Signs
The symptoms of Parry-Romberg syndrome are very characteristic and quickly indicate the disease. The typical atrophy in Parry-Romberg syndrome always affects one half of the face. In addition, the affected person’s lips usually become puckered.
In addition, patients suffer from trigeminal neuralgia, which is accompanied by a sensation of pain in the diseased half of the face. Those affected often lose body weight and exhibit circular hair loss. This is particularly evident in the initial phase of Parry-Romberg syndrome.
The affected persons are mostly comparatively young women. Half-facial atrophy is progressive and affects some or all of the tissues. At the onset of atrophy, the affected areas sometimes develop hyperpigmentation. Hypopigmentation is also possible. Muscles, bones, cartilage and fatty tissue are often also affected by atrophy.
In some cases, the limbs of the patients are also affected by the symptoms of Parry-Romberg syndrome. In addition, some of the patients suffering from Parry-Romberg syndrome suffer from focal epilepsies and migraine attacks. Sometimes the hair falls out on the affected areas of the skin.
Diagnosis & course of disease
When diagnosing Parry-Romberg syndrome, the doctor first conducts an anamnesis with the sick person. In addition to visual examinations of the affected half of the face, the focus is then primarily on imaging procedures. The head is examined using MRI or CT techniques. In addition, biopsies of the affected skin areas are used. The tissue samples are analyzed histologically in the laboratory and contribute to the diagnosis of Parry-Romberg syndrome.
Due to the Parry-Romberg syndrome, those affected suffer from severe discomfort in the face or usually on one side of the face. This can also lead to a significantly increased pain sensation in this half of the face. Those affected often also suffer from inferiority complexes or reduced self-esteem and no longer feel beautiful.
Hair loss occurs, which further reduces the patient’s quality of life. Likewise, the Parry-Romberg syndrome leads to hyperpigmentation, which can also have a negative effect on the aesthetics of the person concerned. Most patients continue to suffer from severe headaches and migraines. It can also lead to epileptic seizures and thus to severe cramps and pain.
Because of the aesthetic problems, most patients also suffer from psychological problems or depression. The symptoms of Parry-Romberg syndrome can be limited with the help of antibiotics or other medication. There are no other complications during the treatment. The symptoms can also be treated relatively well with the help of UV rays. The life expectancy of those affected is not affected by Parry-Romberg syndrome.
When should you go to the doctor?
If the typical atrophy is noticed in one half of the face, a doctor’s visit is recommended. If you have other symptoms typical of Parry-Romberg syndrome, such as muscle, cartilage or bone changes, you must seek medical advice immediately. The Parry-Romberg syndrome occurs in connection with Borrelia, trauma and an autoimmune genesis. If the symptoms described occur in connection with these conditions, it is best to inform the responsible doctor. Parry-Romberg syndrome is diagnosed by a dermatologist or an internist. Treatment is surgical and drug.
The patient must be treated as an inpatient and, even after completion of the initial therapy, consult the doctor to discuss further reconstructive and plastic surgical measures. Migraine, epilepsy and other side effects are treated by neurologists, internists and other specialists who are responsible for the individual symptoms. Since the severe external deformities can also represent a significant emotional burden, psychological treatment is also useful. Parents of affected children should consult a child psychologist.
Treatment & Therapy
The Parry-Romberg syndrome cannot be treated causally, since the exact causes are still largely unknown. Once the atrophy has come to an end, most patients desire cosmetic therapy. Enormous advances have been made in this area today.
Surgical reconstructions of the diseased half of the face can be considered here, so that the symmetry of the face is restored as far as possible. In principle, the course of Parry-Romberg syndrome is self-limiting. The atrophy usually progresses progressively over a period of seven to nine years. In this phase, the disease can neither be stopped nor cured.
In individual cases, however, successes can be seen with different treatment approaches. These include, for example, corticosteroids and other immunosuppressive drugs. Certain antibiotics such as ceftriaxone may also have a positive effect on the course of Parry-Romberg syndrome. It is also of great help to diagnose Lyme disease at an early stage. In addition, therapy with UV-A rays has a positive effect on Parry-Romberg syndrome.
Outlook & Forecast
For unknown reasons, Parry-Romberg syndrome is most common in women under the age of twenty. So far there is no really successful therapy. However, the disease can suddenly come to a standstill after a few years. However, this does not mean a cure. In this respect, the prognosis is only partially positive. After all, the affected half of the face can be surgically restored after the disfiguring disease has stopped.
Parry-Romberg syndrome progresses over the first few years. A normal course of the disease lasts about seven to nine years. In order to alleviate the after-effects for those affected, physicians have recorded more or less good successes with various therapy attempts. In the absence of other alternatives, Parry-Romberg syndrome is treated with corticosteroids, immunosuppressants or antibiotics during the acute phase.
The latter is used in particular when Lyme disease is present or suspected to be the trigger. Treatment with UV-A rays sometimes seems to be successful. If the Parry-Romberg syndrome is no longer active, surgical-reconstructive or plastic-surgical measures are used depending on the extent of the visible damage to the face.
It is possible, for example, to use a pedicled and free fatty tissue plastic. In this way, the atrophied half of the face can be restored, at least visually. Some doctors also use autologous lipo injections or foreign body injections, so-called hydroxyapatite granules. The aim of these measures is to make the disfigured face of those affected more attractive again.
Prevention of Parry-Romberg syndrome is not possible because the pathogenesis of the disease has not been sufficiently researched.
In most cases, those affected with Parry-Romberg syndrome have no special and direct options for aftercare, so that with this disease the focus is on early detection and subsequent treatment of the disease. The earlier the disease is recognized and treated by a doctor, the better the further course of the disease is usually.
Therefore, the affected person should consult a doctor as soon as the first symptoms and signs appear in order to prevent the symptoms from getting worse. Most people affected by this disease are dependent on taking various medications. It is always important to take the right medication and also to pay attention to the right dosage in order to relieve the symptoms permanently and, above all, correctly.
If anything is unclear or if you have any questions, consult a doctor first. Regular checks and examinations of the skin are also very important so that damage to the skin can be detected at an early stage. As a rule, Parry-Romberg syndrome does not reduce the life expectancy of the person affected. Further measures or options for aftercare are not available to the person concerned, although these are usually not necessary.
You can do that yourself
People suffering from Parry-Romberg syndrome need medical attention. Depending on the severity of the disease, individual symptoms can be alleviated independently. Rest helps with migraines and focal epilepsies. The patient should also change their diet to reduce the risk of migraine attacks or epileptic seizures. Proven methods include the ketogenic diet and the raw food diet. In the event of hair loss, a change in diet may also make sense. A hair piece can be worn or a natural hair restorer can be used.
If you have a severe Parry-Romberg syndrome, which also manifests itself in malformations and tissue damage, moderate exercise is part of self-help. The patients should work out a suitable therapy concept together with the doctor and a physiotherapist. The closer the treatment is to the individual complaints, the better the chances of a speedy recovery.
Since the syndrome can persist over a long period of time despite all measures, therapeutic measures are also useful. The sick should, for example, talk to other sufferers or talk to friends and relatives about the condition. The family doctor can provide suitable contact points for talk therapy.