Signs and Symptoms
- Initial facial changes involve the temporal or buccinator muscles
- Disease progressively spreads, resulting in atrophy of skin and underlying structures
- Mouth and nose deviate towards affected side of face
- Process may extend to involve tissues between nose and upper corner of lip, upper jaw, angle of mouth, area around eye and brow, ear, and/or neck
- Hair and skin overlying affected areas may become hyperpigmented or hypopigmented

Skin and Connective Tissues
- Facial changes involve subcutaneous tissue, connective tissue, and muscles of one side of the face
- Cartilage of nose, ear, and larynx can be involved
- Scar resembling a wound made by a sabre may appear in frontal region of scalp
- Hair loss and depressed linear scar may be observed
- Disease may involve neck, trunk, arm, or leg on same or opposite side

Neurological
- Roughly 45% of individuals with Parry-Romberg syndrome have trigeminal neuralgia or migraine
- 10% develop a seizure disorder, typically Jacksonian in nature
- Seizures occur on the opposite side of the affected face
- Abnormalities in gray and white matter of the brain may be detected on MRI scan
- Neurological symptoms usually appear during the first or second decade of life

Ocular
- Recession of eyeball within the orbit is common
- Loss of subcutaneous tissue around the orbit causes eye abnormalities
- Other findings include drooping eyelid, pupil constriction, redness of conjunctiva, and decreased sweating on affected side
- Ocular abnormalities may include ophthalmoplegia, strabismus, uveitis, and heterochromia of the iris
- Signs collectively referred to as Horners syndrome

Oral
- Tissues of mouth commonly involved in Parry-Romberg syndrome
- Dental abnormalities, delayed eruption, and dental root exposure may occur
- Difficulty or inability to normally open the mouth or other jaw symptoms may be present
- Atrophy of one side of upper lip and tongue may be experienced
- Soft palate, gingiva, and teeth may also be affected

Epidemiology:
- Parry-Romberg syndrome occurs randomly and for unknown reasons.
- Prevalence is higher in females than males, with a ratio of roughly 3:2.
- The condition is observed on the left side of the face about as often as on the right side.

History:
- Parry-Romberg syndrome was first described in 1825 by Caleb Hillier Parry and published posthumously by his son Charles Henry Parry.
- It was described a second time in 1846 by Moritz Heinrich Romberg and Eduard Heinrich Henoch.
- German neurologist Albert Eulenburg was the first to use the descriptive title progressive hemifacial atrophy in 1871.

Management:
- Medical management may involve immunosuppressive drugs such as methotrexate, corticosteroids, cyclophosphamide, and azathioprine
- Benefits of medical treatments have not been clearly established due to lack of randomized controlled trials
- Autologous fat transfer or fat grafts may be used to restore normal contour to the face
- Microsurgical reconstruction may be necessary for greater volume defects
- Management aims to alleviate symptoms and improve appearance