Is Parry–Romberg Syndrome an Autoimmune Disease?

Parry-Romberg Syndrome
An autoimmune mechanism is suspected for Parry-Romberg Syndrome (PRS), however, other research also links infectious diseases to the syndrome.

Parry–Romberg syndrome (PRS) is a rare degenerative disease that affects one side of the face called progressive hemifacial atrophy. It causes atrophy (shrinkage) of the subcutaneous tissue, cartilage, fat tissue, and underlying bone structures on one-half of the face.

The exact cause of the disease is unknown, but speculations were made that there might be involvement of autoimmune factors. An autoimmune disease is a condition where the immune system misidentifies healthy cells in a person's body as foreign and destroys them. Numerous studies have suggested that autoimmune inflammatory processes have a role in the development of PRS, and treatment of patients with immunosuppressants has shown some good results. Patients with PRS have a significant rate of autoimmune antibody positivity.

To summarize, although immune responses may play a role in causing PRS in many cases, many other factors may be involved. Some studies even showed that PRS may be linked with infectious diseases because patients with hepatitis B virus and herpes zoster infection had developed the syndrome.

What are the symptoms of Parry–Romberg syndrome?

Parry–Romberg syndrome (PRS) often manifests itself within the first 20 years of life. It is a slowly progressive disease, and the active period may last up to 2 to 10 years. During this period, tissue on half of the face thins or wastes away. Subcutaneous tissue (the tissue beneath the skin) is most commonly involved, followed by the skin and muscles of the face. 

When the illness manifests itself early, the facial skeleton on that side may be affected. This causes facial asymmetry (a distinct look on each side of the face), and the affected side has a sunken-in appearance. The often-reported feature is described as a straight depression running from the brow to the hairline known as the C.

Computed tomography and magnetic resonance imaging may reveal subcutaneous tissue, fat, skin, underlying musculature, salivary glands, and muscular atrophy, and/or malformed skeletal structure.

Generally, the first changes occur to the tissues above the upper jaw (maxilla) or between the nose and upper corner of the lip (nasolabial fold), and they are followed by the angle of the mouth, regions surrounding the eye, brow, ear, and neck.

Muscles in the face may atrophy, and bone loss in the facial bones may occur. The tongue, soft and fleshy portion of the roof of the mouth, and gums may suffer from degeneration.

The affected side's eye and cheek may look sunken, and facial hair may turn white and fall (alopecia). When the illness surrounds the eye, it can cause problems with vision. Furthermore, the skin covering affected regions may become darkly pigmented (hyperpigmented) with patches on the pigmented skin (vitiligo).

PRS may affect other regions of the body along with the face and skin and cause seizures, headache, and trigeminal neuralgia (pain on one side of the face caused by the trigeminal nerve) along with other symptoms.

What is the treatment of Parry–Romberg syndrome?

There is no definitive treatment or cure for Parry–Romberg syndrome (PRS), and there are no therapies that can slow or stop its growth. Patients with PRS have different prognoses. Some patients may not have any development of disability, just slight changes in the appearance of their face.

To restore the lost tissue, reconstructive or microvascular surgery may be required. Surgical intervention is usually done after the exhaustion of the course of illness and changes in the face are developed completely. Most surgeons would advise the patient to wait one or two years before undergoing reconstruction. Other forms of therapy include symptomatic and supportive management such as pain medications and counseling.

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Parry-Romberg Syndrome:

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