Parry Romberg Syndrome
Evidence: Unconfirmed
Names
Progressive hemifacial atrophy (PHA)
Progressive facial hemiatrophy
Idiopathic hemifacial atrophy
Romberg’s syndrome
Description
Parry Romberg Syndrome predominantly affects the skin of the face and is believed to be autoimmune in nature, though the cause is still being researched. Typically, the skin deteriorates gradually over one side of the face, beginning with the upper jaw and the area extending from the nose to the corner of the lip, and then spreading to other parts of the face. In some cases, symptoms are not restricted to only one side of the face and may also occur in the trunk or limbs. Severe cases can spread to the muscles, bones, and cartilage. Patients can expect symptoms to progress over a period of 2 to 20 years, after which time progression plateaus. Treatments have so far been unsuccessful in stopping progression, but they do help manage associated discomfort. Cases that progress over longer periods of time and beginning at a younger age often lead to the most severe symptoms, resulting in facial distortions and nerve disorders.
Prevalence
It is estimated that there are around ~1 case per 700,000 people. 75% of patients are women.
Van der Cruyssen et al (2018)
Typical Age of onset
Starts around 10 years of age, but adult onset is possible.
Van der Cruyssen et al (2018)
Symptoms
Thinning or shrinkage of various tissues of the face (including fat, skin, connective tissues, muscle, and sometimes bone)
Sunken appearance of the face
Depressions in bones of the head
Bald patches
Absence of eyelashes or portions of the eyebrow
Whitening of hair over affected areas
Difficulty chewing or moving the jaw
Misalignment of the jaw and teeth
Curling of the upper lip
Migraine headaches
Seizures on the side of the body not affected by Parry-Romberg Syndrome
Prickling or burning of the face
Episodes of severe pain in face
Displacement of the eyeball farther back in the eye socket than normal
Drooping of the upper eyelid
Different colored eyes
Difficulty closing the eye
Darkening or lightening of the skin over affected areas
Inflammation of the iris or middle part of the eye
