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 randomised 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