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Morphea, also known as localized scleroderma, is defined as a sclerosus skin condition that can extend to the subcutaneous tissue but with no Raynaud's phenomenon or visceral involvement. Some clinical forms may have a functional and aesthetic impact, hence the interest of treatment of early stage inflammation. We here describe the epidemio-clinical, therapeutic and evolutionary features of different forms of morphea, by reporting a series of 24 cases.

Immunologic data collected in 11 children (6 girls and 3 boys under fourteen) presenting with linear scleroderma were analysed in a retrospective study: 2 children presented with superficial linear scleroderma, 6 with monomelic scleroderma, 1 with dimelic scleroderma, 1 with the "en coup de sabre" variety associated with dimelic homolateral involvement, and another with "en coup de sabre" scleroderma combined with facial hemiatrophy: Antinuclear antibodies (ANA) were demonstrated in 9/11 cases (i. e. 81 p. 100). The immunofluorescence staining pattern was homogeneous in all nine with a low titer (less than 250 in 5 of them). ANA to single stranded DNA was present in 1/3. The demonstration of ANA in these 9 children was correlated with deep or extensive sclerosis with muscular involvement in 7. But neither the presence nor the titer of ANA were correlated with the subsequent development of osteoarticular sequelae. The level of total complement appeared to be lowered in 3/8 cases. No renal involvement was demonstrated. Blood tests for circulating immune complexes were positive in 4/8 patients. Skin biopsy for direct immunofluorescence was performed in 6 children and demonstrated immunoglobulin deposits in 4: three had IgM fixation on the dermo-epidermal junction, and one had speckled fixation of IgG on epidermal nuclei (this has not previously been reported in localized scleroderma). There data highlight: a--the high frequency of ANA in linear scleroderma.(ABSTRACT TRUNCATED AT 250 WORDS)

This study involved 20 patients, 13 female and 7 male, with a mean age of 49 years, suffering from localized (6 cases) scleroderma or generalized without Raynaud's syndrome (6 cas), with Raynaud's syndrome (6 cas) or acrosclerosis (2 cases). Respiratory function tests included study of volumes, flow rates, CO transfer (TLCO) and blood gases. There were 38% normal chest X-rays, 58% ventilatory disturbances (predominance of restrictive syndrome and decrease in (TLCO) was found in only 25% of cases. The authors emphasize the extent of pulmonary vascular lesions in these patients.

A combined histological and biomechanical study was performed in patients with localised or systemic scleroderma. The inflammatory reaction was variable in intensity and location, predominating around the superficial vascular plexus, the deep dermo-hypodermal plexus, the paraseptal plexus or in the fasciae. Secondary sclerosis resulted from the lateral fusion of bundles of collagen fibers. Three degrees of intensity of sclerosis were disclosed by our noninvasive technique of tonometry by vertical traction of the skin in vivo. That measurement allowed a follow-up of the evolution of the disease and a control of the therapeutic efficacy.

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.