Parry-Romberg syndrome is a disease that occurs with a very low prevalence in the population. As part of the disease, affected individuals develop progressive atrophy that usually affects 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 the medical community as hemifacial atrophy or progressive facial hemiatrophy. It is a very rarely occurring disease whose exact triggers have not been adequately researched. In Parry-Romberg syndrome, affected individuals suffer atrophy or hemiatrophy, respectively, which is seen on one half of the face. The name of the disease is derived from the two physicians who first scientifically described Parry-Romberg syndrome. They were Caleb Parry and Moritz Romberg. The exact incidence of Parry-Romberg syndrome has not yet been researched. However, previous observations of patients show that Parry-Romberg syndrome affects female patients in the majority of cases. They often have a relatively young age. Approximately three-quarters of cases of the disease are found in women who have not yet completed their second decade of life.
Causes
Currently, no medical research studies are available regarding the exact causes of Parry-Romberg syndrome. The lack of knowledge regarding the pathogenesis of the disease is also due to its rarity. However, conjecture suggests that damage to nerves underlies the unilateral atrophy of the face. This lesion results in a reduced blood supply to the respective half of the face. As a result, atrophy or hemiatrophy of the facial tissue develops due to a lack of oxygen and nutrients. In addition, other potential causes of Parry-Romberg syndrome are under discussion. For example, some researchers believe that certain bacteria, such as Borrelia, are involved in the development of Parry-Romberg syndrome. In addition, autoimmune diseases are being considered as causes of the disease. In many individuals suffering from Parry-Romberg syndrome, the atrophy of one side of the face stops on its own after a certain period of 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, speaks for an involvement of the body’s own defense system. In addition, rheumatoid markers are identified in sufferers in about 30 percent of cases.
Symptoms, complaints, and signs
The symptoms of Parry-Romberg syndrome are very characteristic and quickly point to the disease. In Parry-Romberg syndrome, the typical atrophy always affects one half of the face. In addition, the lips of affected individuals usually become distorted. Patients also suffer from trigeminal neuralgia, which is associated with pain sensation in the affected half of the face. Affected individuals often lose body weight and exhibit circular hair loss. This is most apparent in the early stages of Parry-Romberg syndrome. Affected individuals are usually comparatively young women. Atrophy of the face is progressive and affects either some or all tissues. At the onset of atrophy, the affected areas sometimes develop hyperpigmentation. Hypopigmentation is also possible. Muscle, bone, and cartilaginous and fatty tissues are often also affected by atrophy. In addition, in some cases, patients’ limbs 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 on the affected areas of the skin falls out.
Diagnosis and course of the disease
When diagnosing Parry-Romberg syndrome, the physician first takes a medical history with the affected person. In addition to visual examinations of the affected half of the face, imaging techniques are then the main focus. The head is examined using MRI or CT techniques.In addition, biopsies of the affected skin areas are used. The tissue samples are histologically evaluated in the laboratory and contribute to the diagnosis of Parry-Romberg syndrome.
Complications
Due to Parry-Romberg syndrome, affected individuals suffer from severe facial discomfort, or usually one half of the face. This may further include a marked increase in pain sensation in that half of the face. Those affected also often 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, Parry-Romberg syndrome leads to hyperpigmentation, which can have an equally negative impact on the aesthetics of the affected individual. Most patients also suffer from severe headaches and migraines. Epileptic seizures may also occur, resulting in severe convulsions and pain. Due to the aesthetic discomfort, most patients also suffer from psychological discomfort or depression. With the help of antibiotics or other medications, the symptoms of Parry-Romberg syndrome can be well limited. Further complications do not occur during treatment. The symptoms can also be treated relatively well with the help of UV rays. The life expectancy of the affected person is not affected by Parry-Romberg syndrome.
When should you see a doctor?
When the typical atrophy is noticed in one half of the face, a visit to the doctor is recommended. If there are other symptoms typical of Parry-Romberg syndrome, such as muscle, cartilage, or bone changes, medical advice must be sought immediately. Parry-Romberg syndrome occurs in association with Borrelia, trauma, and autoimmune genesis. If the symptoms described occur in association with these conditions, it is best to inform the appropriate physician. Parry-Romberg syndrome is diagnosed by a dermatologist or internist. Treatment is surgical and medicinal. The patient must be treated as an inpatient and must also consult with the physician after initial therapy is completed to discuss further reconstructive and plastic surgical procedures. Migraine, epilepsy and other accompanying symptoms are treated by neurologists, internists and other specialists responsible for the individual symptoms. Since the severe external deformities can also represent a considerable psychological burden, psychological treatment is also advisable. Parents of affected children should consult a child psychologist for this purpose.
Treatment and therapy
Parry-Romberg syndrome is not amenable to causative therapy, because the exact factors that cause it are still largely unknown. Once the atrophy has come to an end, most patients desire cosmetic therapy. Enormous progress has been made in this area nowadays. Surgical reconstructions of the diseased half of the face are considered, so that the symmetry of the face is restored as far as possible. Basically, Parry-Romberg syndrome is self-limiting in its course. 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, success has been achieved with various 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. In addition, it is of great help to diagnose an existing Lyme borreliosis at an early stage. In addition, therapy with UV-A rays takes a positive influence on Parry-Romberg syndrome.
Outlook and prognosis
Parry-Romberg syndrome mostly affects women younger than twenty years of age for unknown reasons. To date, there is no truly successful treatment. However, the disease may suddenly stop after a few years. However, this does not mean a cure. In this respect, the prognosis is only conditionally positive. At least, the affected half of the face can be surgically restored after the disfiguring disease has stopped. Parry-Romberg syndrome progresses during the first few years. A normal course of the disease takes about seven to nine years.To ease the sequelae for those affected, physicians have had more or less good success with various therapeutic attempts. For example, in the absence of other alternatives, Parry-Romberg syndrome is treated during the acute phase with corticosteroids, immunosuppressants or
antibiotics. The latter is used especially when Lyme disease is present or suspected as a trigger. Treatment with UV-A radiation also sometimes seems to be successful. If 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. For example, the use of pedicled and free fatty tissue plastic surgery is possible. This can restore the atrophied half of the face, at least visually. Some physicians also use autologous lipo-injections or foreign body injections, so-called hydroxyapatid granules. The goal of these measures is to make the affected person’s disfigured face more presentable again.
Prevention
Prevention of Parry-Romberg syndrome is not possible because the pathogenesis of the disease has not been sufficiently studied.
Follow-up
In most cases, the person affected by Parry-Romberg syndrome does not have any special and direct options for aftercare, so the main focus in this disease is early detection with subsequent treatment of the disease. The earlier the disease is detected and treated by a physician, the better the further course of the disease usually is. Therefore, the affected person should already consult a doctor at the first symptoms and signs in order to prevent a further worsening of the symptoms. Most sufferers are dependent on taking various medications for this disease. It is always important to ensure that the medication is taken correctly and also the correct dosage in order to alleviate the symptoms permanently and, above all, correctly. If there are any uncertainties or questions, a doctor should be consulted first. Furthermore, 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 affected person. Further measures or possibilities of an aftercare are not available to the affected person thereby, whereby these are mostly also not necessary.
What you can do yourself
People suffering from Parry-Romberg syndrome need medical help. Depending on the severity of the condition, individual symptoms can be relieved independently. In case of migraine as well as focal epilepsies, sparing helps. Patients should also change their diet to reduce the risk of migraine attacks or epileptic seizures. Proven methods are the ketogenic diet and the raw food diet. In cases of hair loss, a change in diet may also be useful. This can be accompanied by wearing a hairpiece or using a natural hair restorer. In the case of severely pronounced Parry-Romberg syndrome, which is also manifested by malformations and tissue damage, moderate exercise is part of self-help. Patients should work out a suitable therapy concept together with their doctor and a physiotherapist. The more closely the treatment is oriented to the individual complaints, the higher the chances of a rapid recovery. Since the syndrome can persist over a long period despite all measures, therapeutic measures are also useful. Sufferers should, for example, talk to other sufferers or discuss the condition with friends and relatives. The family doctor can provide suitable points of contact for talk therapy.
