Progressive hemifacial atrophy (Parry-Romberg Syndrome)

Progressive hemifacial atrophy, also known as Parry-Romberg Syndrome, is an uncommon degenerative and poorly understood condition. It is characterized by a slow and progressive atrophy affecting one side of the face. The incidence and the cause of this alteration are unknown. A cerebral disturbance of fat metabolism has been proposed as a primary cause. This can be result of a trophic malformation of cerebral sympathetic nervous system. Possible factors that are involved in the pathogenesis are trauma, viral infections, heredity, endocrine disturbances, and autoimmunity, among others. Characteristically, atrophy progresses slowly for several years and, soon after, it become stable. The purpose of this work is, through the presentation of a clinical case, to accomplish a literature review concerning general characteristics, etiology, pathophysiology, differential diagnosis, and treatment of progressive hemifacial atrophy.