[Linear scleroderma in children (apropos of 27 cases)]

Abstract

Twenty-seven cases of linear morphoea are reported and compared with 218 cases collected from the literature. The various parameters studied, including clinical features and laboratory results, were identical in both series. The aetiology of linear morphoea is unknown, even though injuries or fever have been noted as triggering factors in 20 p. 100 of the cases. Linear morphoea is a childhood disease: it begins at the age of 7 or 8 and predominates in females (63 p. 100 of the cases). Its onset is marked by the abrupt occurrence of sclerosis in most cases, although a solitary morphoea or a trophic plaque may precede the disease proper. In our series, muscular or articular involvement appeared from the start in 40 p. 100 of the patients at the same time as the initial cutaneous lesions. The active stage lasts 3 years and sometimes longer. It is characterized by extension (37 p. 100) or both extension and multiplication (63 p. 100) of the initial lesions. Regional complications aggravate linear morphoea and are more frequent in patients whose lesions extend and multiply. Their incidence was lower among the published cases than in our series. Depending on the author, retractile myositis is present in 37 to 59 p. 100 of the cases, joint stiffness in 18 to 40 p. 100 and shortening of a limb in 10 to 22 p. 100. These complications often regress incompletely, leaving sequelae which persist in the steady state in 75 p. 100 of the patients. Antinuclear antibodies, present in 37 p. 100 of the cases, are either of the homogeneous or of the speckled type. Jablonska has met them more frequently (50 p. 100), and they were often of the speckled type. The significance of these antibodies in linear morphoea is unclear since they appear inconstantly and later than clinical signs. The skin lesions associated with linear morphoea show that the other forms of scleroderma--i.e. plaque morphoea, erythematous atrophic or dyschromic plaques and guttate scleroderma--belong to the same family. The same associations are found in frontoparietal "coup de sabre" scleroderma. The treatment of linear morphoea is not yet standardized. At the moment, the best and most regular results are obtained with systemic corticosteroids and local physiotherapy.