![]() Ocular examination and skin examination showed no abnormality with any evidence of lymphadenopathy. Thinning of the lips on the affected side with exposition of the teeth due to the corner of the mouth pulled to left side. There was an evident loss of underlying fat, muscle and subcutaneous tissue giving a sunken in appearance. Head and neck examination confirmed the asymmetry with flattening, hyperpigmented, parchment like overlying skin. Right side of the face appeared normal with marked facial asymmetry noted on left side. On general physical examination patient was conscious, oriented, a febrile, general condition was fair and vital signs were stable. Her medical and family history was non contributory. The patient had not sought any consultation earlier, for the facial disfigurement. However it was not associated with any other symptoms except for the limited mouth opening which the patient experienced gradually as the condition progressed. Progressive atrophy on the left half of the face was initially noticed at the age of 8 years as an area of hyper pigmentation on the left malar area which gradually progressed with resultant atrophy of the underlying fat and subcutaneous tissue resulting in the present disfigurement (Fig. 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” ( 9).Ī 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. Parry Romberg syndrome is usually accompanied by neurological complications like trigeminal neuralgia, migraine, seizures and also accompanied by ocular complication of Horner’s syndrome ( 8). However 5-10% of cases being bilateral have also been reported ( 6). The disease is usually limited below the forehead region though involvement of the scalp along with loss of hair or premature graying can also be seen. Unilateral facial involvement is common and generally follows the pattern of sensory innervations of one or all three trigeminal nerve dermatomes ( 7). However 10% of the cases have been reported with entire half of the body involvement ( 6). The condition is most often found in female population compared to male in the ratio 3:2 and has predilection for left side of the face. The atrophy is insidious in onsfiget occurring in the first two decades of life lasting for 2-10 years followed by burning out of the atrophy with resultant stability ( 5). Other possible factors include trauma, viral infections, endocrine disturbances, heredity and auto immunity are believed to be involved in the pathogenesis of the condition ( 4). ![]() Waternberg concluded that hyperactivity sympathetic nervous system, due to disturbed central regulation leads to trophism of underlying fat and subcutaneous tissue ( 3). However immune mediated processes are primarily considered. Even after the condition being recognized for more than 150 years the exact incidence and etiology remains unknown. Eulenberg coined the term “progressive facial hemiatrophy” in 1871 ( 2). ![]() First it was described by Caleb Hillier Parry and later on by Moritz Henirich Romberg who described it as a syndrome ( 1). 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. Key words:Parry Romberg syndrome, progressive facial hemiatrophy, morphea, localized scleroderma. 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. Parry Romberg Syndrome is an invalidating lesion that may be associated with different neurological, cutaneous, ocular, dental and autoimmune abnormalities. The syndrome overlaps with “en coup de sabre” morphea, with an ill defined relationship existing between the two. Apart from the multifactorial etiology proposed, the possible primary cause is mainly attributed to the cerebral disturbance of the fat metabolism. The exact incidence and etiology towards the syndrome remains unclear. 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. ![]()
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