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Parry-Romberg syndrome
  1. Jon Stone
  1. Consultant Neurologist and Honorary Senior Lecturer, Department of Clinical Neurosciences, Western General Hospital, Crewe Road, Edinburgh EH4 2XU, UK; Jon.Stone@ed.ac.uk

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    Parry-Romberg syndrome, which is also called progressive facial hemiatrophy, overlaps with a condition known as linear scleroderma “en coup de sabre”. It is a rare, acquired, neurocutaneous syndrome of unknown aetiology. The principle features are atrophy of the soft tissues, and sometimes the bone, on one half of the face or forehead without facial weakness. Sometimes the atrophy extends to the limbs, usually on the same side, and there may be various ophthalmological and neurological complications. As a neurologist you should know about this syndrome because you will probably encounter at least one or two cases during your career, perhaps to assess new onset facial asymmetry, hemi-masticatory spasm, or because a patient already with the diagnosis has developed epilepsy or difficult migraine. Patients with Parry-Romberg syndrome present to various other clinics—especially dermatology, plastic surgery, and rheumatology.

    CLINICAL FEATURES

    The main feature is hemiatrophy of facial tissues, typically fat, but variably skin, other connective tissue, and sometimes bone (fig 1). The jaw, mouth, cheek, or forehead may all be affected. Patients range in severity from those with barely perceptible asymmetry to severe disfigurement. The range of clinical features is outlined in the table. Where the atrophy meets normal tissue on the other side of the face, it may produce a “line”. Around 25% of patients with facial hemiatrophy have a more definite vertical or diagonal “line” on their forehead as a result of cutaneous sclerosis (rather than atrophy of the deeper tissues) (fig 2). These lines tend to follow “Blaschko’s lines” which are the diagonal/ vertical lines on the forehead, …

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