Idiopathic acquired progressive left facial hemiatrophy (Parry-Romberg syndrome) in a 21-year-old man in semi-urban, south-west Nigeria

Abstract

Idiopathic progressive hemifacial atrophy, or Parry-Romberg syndrome, is a rare entity, seldom described in medical texts. Though first described in 1825, as yet there are no clear-cut diagnostic criteria. It is of interest mainly because of the numerous features with which it may be associated, which are largely in the central nervous system, and, more importantly, because it can easily be confused with localised scleroderma en coup de sabre. Some classify it as one of the trophoneuroses; others believe it is a variant of localised scleroderma. Most of the sufferers present because of the attendant cosmetic loss from the facial disfigurement. Treatment is multifactorial, and includes facial reconstructive surgery and immunosuppressants. Here we present the case of a 21-year-old Nigerian with idiopathic progressive left hemifacial atrophy. To the best of our knowledge, it is probably the first case from Nigeria to be reported in literature.