How Fast Does Parry-Romberg Syndrome Progress?

Medically Reviewed on 1/12/2023
An autoimmune mechanism is suspected for Parry-Romberg Syndrome (PRS), however, other research also links infectious diseases to the syndrome.
An autoimmune mechanism is suspected for Parry-Romberg Syndrome (PRS), however, other research also links infectious diseases to the syndrome.

Parry-Romberg syndrome is a rare acquired disorder that is seen in the first or second decade of life. It progresses slowly before entering the stable phase.

The condition is characterized by the thinning or shrinkage of various tissues of the face, including fats, skin, or connective tissue, muscles, and bone. The condition typically affects one side of the face; however, it can sometimes affect both sides as well.

Parry-Romberg syndrome is a rare condition seen in 1 out of 250,000 people and affects girls more than boys.

What causes Parry-Romberg syndrome?

The exact cause of Parry-Romberg syndrome is unknown, but the following few factors are thought to increase the risk of the condition:

  • Autoimmune diseases
  • Bacterial or viral infection
  • Inflammation of the brain and meninges
  • Abnormalities in the nervous system
  • Trauma

Very rarely, the condition may be genetically inherited, but no proper evidence shows the relation between genetics and Parry-Romberg syndrome.

More studies are required to know the proper cause of the Parry -Romberg syndrome. The cause of the syndrome may also vary from person to person, and the development of the condition may require the simultaneous presence of various factors.

What are the signs and symptoms of Parry-Romberg syndrome?

Parry-Romberg syndrome's most common symptoms include thinning or shrinkage of the skin, soft tissues, muscles, and bones. The shrinkage of muscles and skin results in some evident physical abnormalities or deformities, such as:

  • Abnormal shifting of nose and mouth to one side of the face
  • The eyes, face, and facial skin may show a sunken appearance
  • Ears in the affected individual may be small and appear abnormal, protruding from the face due to loss of supporting tissue in the skin
  • Changes in skin color (hyperpigmentation or hypopigmentation)
  • Hair loss or hair turning white, loss of eyelashes or a portion of eyebrows
  • Facial bone or muscle loss
  • Abnormalities in the mouth, such as trismus (difficulty opening the mouth) and teeth (misaligned teeth)

Systemic abnormalities are also noticed, such as:

How is Parry-Romberg syndrome diagnosed?

The following are the ways to diagnose Parry-Romberg syndrome:

  • Physical and general examination
  • Complete medical history
  • Imaging tests that include
    • CT scan: This takes pictures of internal organs using X-rays from different angles
    • MRIs: Use magnetic and radio waves to take pictures

How to treat Parry-Romberg syndrome

Parry-Romberg syndrome mostly affects the eyes, nose, ears, and mouth, which causes difficulties in vision, hearing, speech, and dental functions. Therefore, it is very important to treat the condition.

The treatment of Parry-Romberg syndrome typically includes surgical procedures. Reconstructive surgery is done, which involves moving the fat and soft tissues from other sites to where it is needed. More extensive procedures are done to treat bone deformities.

Medically Reviewed on 1/12/2023