Abstract
Parry Romberg syndrome(PRS) is a rare acquired poorly understood neurocutaneous syndrome of unknown etiology characterized by slow progressive atrophic changes commonly affecting one half of the face. The exact incidence and etiology towards the syndrome remains unclear. Apart from the multifactorial etiology proposed, the possible primary cause is mainly attributed to the cerebral disturbance of the fat metabolism. The syndrome overlaps with “en coup de sabre” morphea, with an ill defined relationship existing between the two. Parry Romberg Syndrome is an invalidating lesion that may be associated with different neurological, cutaneous, ocular, dental and autoimmune abnormalities. This report presents one rare case of 22 years old female patient with Parry Romberg syndrome associated with localized scleroderma, accompanied by a brief review of literature with classical clinical, radiographic, histological findings and the treatment of progressive hemifacial atrophy. Key words:Parry Romberg syndrome, progressive facial hemiatrophy, morphea, localized scleroderma.
Highlights
Parry Romberg syndrome commonly known as progressive facial hemiatrophy is a very rare degenerative condition characterized by a slowly progressive but self limited unilateral atrophy of the face affecting variably the skin, subcutaneous fatty tissue, muscle, connective tissue and bone
Sometimes an ill defined conflicting relationship exists between PRS and linear scleroderma leading to the controversy and confusion over whether progressive facial hemiatrophy is a distinct disease or a form of linear scleroderma
Linear scleroderma is a subtype of localized scleroderma with unknown etiology that characteristically involves sclerosis confined to the skin
Summary
Introduction Parry Romberg syndrome commonly known as progressive facial hemiatrophy is a very rare degenerative condition characterized by a slowly progressive but self limited unilateral atrophy of the face affecting variably the skin, subcutaneous fatty tissue, muscle, connective tissue and bone. The relationship between linear scleroderma and progressive facial hemiatrophy is not clearly understood with some patients presenting a demarcation line between normal and abnormal skin, known as “coup de sabre” [French term which means “cut of the sword”] (9). Case Report A 22 years old female patient reported to our department with a complaint of facial disfigurement on the left side of the face which gradually progressed over the past 14 years.
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