A rare condition with an unclear etiology, Parry-Romberg syndrome (PRS) is characterized by progressively progressing hemifacial atrophy that affects the facial subcutaneous tissue, skeletal muscle, cartilage, and bone. Parry-Romberg syndrome symptoms can range in severity from minor to severe depending on the individual. The adnexa, orbit, and globe all exhibit a broad spectrum of symptoms and indications. As a result of the facial atrophy seen in PRS, changes to the periocular structures take place. The skin may thin out, the facial bones may shift, and the softer tissue (muscle and fat) slowly diminishes with time.
A disorder known as Parry-Romberg syndrome, also known as Romberg syndrome or gradual facial hemiatrophy, causes the tissue on one side of the face to progressively deteriorate. Rarely, the face on both sides is impacted. On the same side of the body as the face shrinkage, limb atrophy might occur in certain persons. Parry-Romberg syndrome symptoms might vary greatly from person to person in terms of their severity. Some individuals may also experience neurological abnormalities, ocular or dental abnormalities, or additional symptoms. Although it can happen in adults, Parry-Romberg syndrome typically manifests itself in the first or early second decade of life.
The majority of Parry-Romberg syndrome sufferers begin to exhibit symptoms before the age of 20. Parry-Romberg syndrome appears to happen randomly and for unknown reasons (sporadically), but its precise cause is unknown. One in 250,000 persons is considered to be affected by Parry-Romberg syndrome, but the actual frequency is unknown because the disease is frequently misdiagnosed. Beginning in childhood, Parry-Romberg syndrome often affects the lower part of the face. Rarely, the face on both sides is impacted.
Parry-Romberg syndrome appears to happen at random and for unknown causes (sporadically), but its cause is unknown. Numerous theories have been put forth to explain how the disorder came to be, including abnormal sympathetic nervous system development or inflammation, viral infections, meningoencephalitis, inflammation of the brain and meninges covering the brain, trauma, abnormal blood vessel formation (angiogenesis), and autoimmune disease. One particular idea holds that an autoimmune response is brought on by infection in the nerves supplied by skin and fat. When the immune system of the body unintentionally targets healthy tissue, autoimmune diseases result. Antibodies are specialized proteins made by the immune system that work to eliminate foreign substances like bacteria, viruses, and poisons.
Some sufferers of Parry-Romberg syndrome may have a past history of head or face injuries. This finding may be coincidental because many people with Parry-Romberg syndrome don’t have a previous record of trauma. To ascertain whether trauma contributes in any way to the emergence of Parry-Romberg syndrome, more study is required. Rarely, some people with Parry-Romberg syndrome have ancestors who had an asymmetrical faces. However, there is no concrete proof that a genetic component contributes to the onset of Parry-Romberg syndrome. Additionally, there is no proof that the disease can be transmitted to offspring.
Initially, facial alterations typically affect the parts of the face that the buccinator or temporal muscles cover. The skin and its adnexa, along with supporting subcutaneous components such as collagen, connective tissue (fat, fascia, cartilage, bones), and/or muscles on one side of the face, atrophy as the disease progresses gradually from the initial spot. Usually, the lips and nose are turned towards the side of the human face that is affected.
The procedure may eventually encompass the tissues in the area surrounding the eye and forehead, the top portion of the lip, the top of the jaw, the opening of the mouth, the ear, and/or the cervical region. The illness frequently starts with a small area of scleroderma on the scalp’s frontal region, which is followed by hair loss and the emergence of a depressed horizontal scar that runs across the middle of the face on the side that is affected. Because it mimics the scar left by a saber wound and is similar to the scar seen in frontal linear scleroderma, this scar is known as a “coup de sabre” lesion.
When a patient simply exhibits facial asymmetry, the diagnosis can be made purely on the basis of the patient’s medical history and physical examination:
- A thorough examination of your child’s whole medical history.
- A thorough physical examination that looks for indicators of the loss of skin, fat, muscle, and bone.
- Specialized testing, such as a CT scan or MRI (a painless imaging procedure that produces precise images of the internal organs and structures of the body).
Surgery is still the primary therapy for Parry-Romberg syndrome because medical treatment has mainly failed. The majority of surgeons advocate delaying therapy until the disorder has “burned out.” Fat transfer utilizing injection techniques is typically used to treat minor abnormalities, and it may be necessary to do the procedure more than once. You might also think about using dermal fillers like polylactic acid and sodium hyaluronate. The chosen course of treatment for patients with more severe Parry-Romberg syndrome is a microsurgical soft tissue transplant from another part of the body. To get the best results for some patients, the two methods may be combined.
The safest and most successful method for treating any condition is homeopathy. As they are created using natural ingredients, homeopathic treatments are free of adverse effects. Successful treatment of the Parry-Romberg syndrome is made possible by homeopathic therapy.
- Cadmium sulphuratum
- Aconitum napellus
Treatment is tailored to the patient’s unique symptoms and frequently involves a team of professionals to address the range of symptoms that a patient may experience. Additionally, because the condition frequently stabilizes on its own, it is challenging to determine whether a therapy was effective. Although systemic drugs used to treat linear sclerodermas, such as antimalarials, methotrexate, a steroid medication, tetracycline, anticonvulsants, and cyclophosphamide, are frequently used to treat PRS, there are no randomized controlled trials for this condition.