Parry-Romberg syndrome, also known as progressive hemifacial atrophy, is a rare condition that causes the deterioration of tissue in a person’s face. Typically, this condition only affects one-half of the face, although it can affect other areas.

Parry-Romberg syndrome affects the soft tissue and skin of a person’s face, causing it to atrophy. Atrophy is when body tissue shrinks or wastes away. Parry-Romberg syndrome is a rare disorder, affecting approximately 1 in 250,000 people. The exact frequency of the condition is unknown as it often goes undiagnosed or misdiagnosed.

In this article, we will discuss Parry-Romberg syndrome, including its symptoms, causes, and treatments.

A person with Parry-Romberg syndrome.Share on Pinterest
Design by Medical News Today; photography by George, Renu; George, Anju; Kumar, T. Sathish1. Update on Management of Morphea (Localized Scleroderma) in Children. Indian Dermatology Online Journal: Mar–Apr 2020 – Volume 11 – Issue 2 – p 135-145 doi: 10.4103/idoj.IDOJ_284_19. CC BY-NC-SA 4.0 and Mehlman A, Patel J, Bitetzakis C, Berlowitz M. Multi-vessel coronary artery aneurysms in a patient with Parry Romberg syndrome: a case report. Eur Heart J Case Rep. 2019;3(3):ytz103. Published 2019 Jul 4. doi:10.1093/ehjcr/ytz103. CC BY-NC 4.0.

Parry-Romberg syndrome is a rare condition that causes the soft tissues of a person’s face to slowly atrophy. It generally affects one side of a person’s face.

According to the National Institute of Neurological Disorders and Stroke (NINDS), Parry-Romberg syndrome usually affects the left side of the face. However, information from the National Organization for Rare Disorders (NORD) notes that there are rare cases of Parry-Romberg syndrome affecting both sides of a person’s face.

Due to difficulties in diagnosing the condition, the incidence of Parry-Romberg syndrome is unknown. However, estimates suggest that it may affect as many as 1 in 250,000 people in the general population. Evidence also indicates that Parry-Romberg syndrome affects more females than males.

Parry-Romberg syndrome usually develops in children and adolescents, with most people experiencing symptoms before the age of 20 years. However, researchers have identified the condition in infants and people over 50 years old.

The name of the condition derives from Caleb Parry, Moritz Romberg, and Eduard Henoch, who were the physicians that originally described the condition in medical literature.

Association with scleroderma

A 2015 review notes that Parry-Romberg syndrome is often thought to be a subtype of localized scleroderma. Localized scleroderma is a condition that causes a person’s skin to thicken. It occurs when a person’s body produces too much collagen. Collagen is a protein that plays an important part in the function and structure of skin and connective tissue.

Some people who have Parry-Romberg syndrome develop a vertical or diagonal line on their foreheads where the atrophied tissue meets unaffected tissue, known as a linear scleroderma “en coup de sabre” (LSCS). The “en coup de sabre” is French for “cut from a sword.”

Approximately one-third of people with linear scleroderma on their head or neck also have Parry-Romberg syndrome. The exact relationship between linear scleroderma and Parry-Romberg syndrome is currently unclear. Some researchers consider the conditions to effectively be the same disorder while others consider them to be separate conditions with considerable overlap.

The cause of Parry-Romberg syndrome is currently unknown. Scientists have various theories, such as:

  • unusual development
  • inflammation of the sympathetic nervous system (SNS)
  • viral infections
  • inflammation of the brain and its membranes
  • trauma
  • abnormalities of blood vessel formation
  • problems with the immune system

Some scientists believe Parry-Romberg syndrome may occur as a result of inflammation in the nerves of fat and skin. This may cause an autoimmune response. Autoimmune conditions occur when a person’s immune system mistakenly attacks healthy tissue. Although there is evidence to suggest that autoimmune inflammation occurs in Parry-Romberg syndrome, scientists are unsure if this is the main cause of the condition.

Evidence suggests that a person may be more likely to develop Parry-Romberg syndrome if they are female and under 20 years of age. However, the condition can affect people of all ages and genders.

The characteristic features of Parry-Romberg syndrome include the atrophy of soft tissue on one side of a person’s face. This can lead to a person having a sunken, uneven appearance. Symptoms of Parry-Romberg syndrome can vary in severity from person to person. These symptoms usually progress over 2–10 years, then stabilize.

Symptoms of Parry-Romberg syndrome can begin in the upper jaw, or between the nose and upper corner of the lip. Symptoms can then spread to the:

  • corner of the mouth
  • areas around the eye
  • brow
  • ear
  • neck
  • tongue
  • soft and fleshy part of the roof of the mouth
  • gums
  • eye
  • cheek

Other possible symptoms of Parry-Romberg syndrome include:

  • whitening of facial hair
  • hair loss, or alopecia
  • darkening pigmentation of the skin over the affected areas
  • unpigmented skin
  • seizures
  • severe facial pain
  • muscle atrophy
  • bone loss
  • retina and optic nerve issues
  • a small or misshapen ear
  • atrophied skin and soft tissue in the arm, trunk, or legs on one side of the body
  • atrophy of internal organs
  • migraines
  • abnormal facial sensations, such as burning or prickling
  • jaw spasms
  • weakness on one side of the body
  • a drooping upper eyelid
  • different colored eyes
  • difficulty closing the eye
  • double vision
  • anxiety
  • depression

A healthcare professional may diagnose a person with Parry-Romberg syndrome by:

  • checking for characteristic symptoms
  • putting together a detailed patient history
  • carrying out a clinical evaluation
  • performing different specialized tests

The tests that a healthcare professional uses to diagnose a person with Parry-Romberg syndrome may depend on the person’s symptoms.

A doctor can use an MRI scan for people who have neurological symptoms. An MRI uses magnetic fields and radio waves to scan a person’s body and produce a cross section of their body tissues and organs. NORD states that between 10–20% of people with Parry-Romberg syndrome may have changes on their brain MRI scans. These changes can include atrophy or inflammation of the brain on the affected side.

There is currently no cure for Parry-Romberg syndrome, and doctors cannot stop its progression. However, treatment options can help a person manage their symptoms.

The treatment required for Parry-Romberg syndrome can depend on a person’s symptoms. A person may require a number of different specialists to treat their condition. These specialists may include:

  • pediatricians
  • internists, who specialize in internal organs and body systems
  • surgeons, including plastic surgeons
  • dentists
  • dermatologists, who specialize in skin conditions
  • ophthalmologists, who specialize in eye conditions
  • neurologists, who specialize in conditions of the brain and nervous system

Doctors generally wait to perform surgery until once a person’s condition stops developing. This allows the surgeon to determine the amount of surgery a person needs. This is why a surgeon may wait until a younger person’s skull and face are no longer growing before performing surgery. A surgeon may also wait 1 or 2 years after symptoms subside before performing surgery.

Surgery for Parry-Romberg syndrome may involve:

  • microvascular surgery
  • muscle or bone grafts
  • fat or silicone injections
  • skin flap grafts

People who have conditions associated with Parry-Romberg syndrome may also receive medication to treat their symptoms. If a person has severe, progressive Parry-Romberg syndrome, a medical professional may suggest using immunosuppressant medications.

However, scientists are currently unsure if immunosuppressants are effective for Parry-Romberg syndrome. Further research is necessary to determine the effectiveness and long-term safety of their use for treating the condition.

The prognosis for a person with Parry-Romberg syndrome can vary depending on the severity of their symptoms. If a person has a mild case of the condition, they may experience cosmetic effects, but not the more painful ones.

There is no evidence to suggest that Parry-Romberg syndrome influences a person’s life expectancy. A person with the condition can speak with a doctor about the possibility of any possible complications.

Parry-Romberg syndrome is a rare condition that typically causes atrophy of the soft tissue on one side of a person’s face. Symptoms can slowly develop over a number of years. Parry-Romberg is more common in females and people under 20 years of age. However, it can affect people of all ages and genders.

Parry-Romberg syndrome can vary in severity. Some people who have the condition may have cosmetic effects while others may experience symptoms, such as seizures or headaches.

Currently, there is no cure for Parry-Romberg syndrome, and no one can stop the progression of the condition. However, treatments are available to help manage symptoms. Additionally, there is no evidence that Parry-Romberg syndrome affects a person’s life expectancy.