Facial Hemiatrophy in a Patient with Systemic Scleroderma

Abstract

A 33-year-old woman with right-sided facial hemiatrophy associated with the acrosclerotic type of systemic scleroderma is described. Symptoms of systemic scleroderma were observed more markedly in the right side.

Progressive hemifacial atrophy: historical review and actual features related to a new classification of facial hypoplasia and atrophy

“…The rare association between diseases of autoimmune basis and hemifacial atrophy could be coincidental or secondary to a slowly progressive autodistruptive process, as strongly suspected for connective tissue diseases [61,69,84,92]; and it must be differentiated from the fibrotic atrophy following panniculitis by Lupus and other connective tissue disease whose onset shows large, painful, nodular or diffuse infiltrating chronic inflammation of deep subcutaneous tissue with fatty necrosis and degeneration greater than that generally reported in the autoimmunity connective tissue diseases [35,52,89]. Therefore, although Parry-Romberg, Multifocal Scleroderma and Autoimmune Diseases are distinct entities of PHA, there might be a line of union: a local or general neurodystrophy or any reduced vitality of peripheral tissues, apparently silent in most affected people, may however appear in relation to stress; but, if a subclinical autoimmune disorder suddenly overthrows the precarious state of a latent or slowly degenerative neurotic or tissue dystrophy, then a severe atrophic disease could break out with several pathologic implications.…”

“…The association with autoimmune diseases, important in order to study the etiopathogenesis of hemifacial atrophy, has been reported specifically with systemic scleroderma [61], with DLE and SLE [84], with rheumatoid arthritis [16,69,84] and arthromyositis [77].…”

Progressive facial hemiatrophy complicated by sclerodactyly, Raynaud’s phenomenon, anti-ribonucleoprotein antibody, and trigeminal nerve disturbance

“…[3][4][5][6][7][8][9] The coexistence of progressive facial hemiatrophy and systemic scleroderma was also reported. 10,11 The close association between progressive facial hemiatrophy and scleroderma was supported by results of biochemical analyses of collagen and glycosaminoglycans of the skin from patients with progressive facial hemiatrophy. 20 Dermatan sulfate/hyaluronic acid ratios were elevated in skin from patients with progressive facial hemiatrophy, which was consistent with findings in patients with morphea or systemic scleroderma.…”

Progressive Hemifacial Atrophy with Localized Scleroderma

“…PHA was first described by Parry in 1825 and Rom berg in 1846 [ 15], Since that time, there have been many opinions on Parry-Romberg's PHA as a distinct noso logic entity or a form of scleroderma [4], Wartenberg [15], in his extensive review, suggested the association of this condition with scleroderma and this opinion has been supported by other authors [12,13]. However, some of them concluded that scleroderma is a definite entity [2,3].…”

“…Muscle, cartilage and bone may also be involved [8], It is known by the eponym, Parry-Romberg's disease, and occasionally termed 'localized scleroderma' by some authors [12,15].…”