Preferred Label : Progressive hemifacial atrophy;
ICD-11 definition : Progressive facial hemiatrophy (Parry–Romberg syndrome) is a rare acquired disorder
of unknown aetiology with onset most commonly during childhood. Progressive atrophy
of the skin, soft tissues and bones of one half of the face may be accompanied by
ocular, dental and neurological problems. Its relationship to linear morphoea of the
face and scalp (morphoea en coup de sabre) has not been fully elucidated. The former
tends not to involve the scalp and forehead and in the latter there is more obvious
cutaneous sclerosis, though the two have been reported to co-exist.;
ICD-11 synonym : Facial hemiatrophy; Hemifacial atrophy; Parry-Romberg syndrome;
ICD-11 inclusion : Parry-Romberg syndrome;
Origin ID : 1238236853;
Currated CISMeF NLP mapping
Progressive facial hemiatrophy (Parry–Romberg syndrome) is a rare acquired disorder
of unknown aetiology with onset most commonly during childhood. Progressive atrophy
of the skin, soft tissues and bones of one half of the face may be accompanied by
ocular, dental and neurological problems. Its relationship to linear morphoea of the
face and scalp (morphoea en coup de sabre) has not been fully elucidated. The former
tends not to involve the scalp and forehead and in the latter there is more obvious
cutaneous sclerosis, though the two have been reported to co-exist.
