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In a young girl with localized scleroderma a circumscribed area of lysis developed in the calcaneus beneath the involved skin and soft tissues of the foot. A biopsy revealed vascular changes characteristic of scleroderma associated with infarction and severe resorption of the bone. A geographic pattern of bone destruction in a child with localized scleroderma has not previously been reported.
Skin biopsies of uniform location and surface area (7 mm diameter) were obtained from the extensor aspect of the forearm of 147 patients with progressive systemic sclerosis (PSS) (107 with diffuse scleroderma, 40 with the CREST syndrome variant) and 58 individuals with normal skin. After careful removal of all subcutaneous fatty tissue, the skin cores were weighed and their water and hydroxyproline content determined. Despite recent claims to the contrary, it was found that there is a marked and highly significant increase in the thickness of the skin during the indurative phase of PSS, and that this is associated with a proportionate increase in total dermal collagen content. A similar degree of thickening was found in the skin of patients with eosinophilic fasciitis and acromegaly. A close correlation was observed between clinical estimation of the degree of skin thickening and the weight of the skin biopsy cores. Change in the weight of skin cores was observed during the course of illness of the patients with PSS and may serve as a useful measurement of alteration in the degree of skin thickening.
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Localized scleroderma in children includes morphea mainly on the trunk and linear scleroderma on the limbs, scalp, and face. Progressive systemic sclerosis is very rare in children and change from localized to progressive disease is extremely rare. Laboratory abnormalities occur with localized scleroderma, including eosinophilia, positive antinuclear factor (ANF), and increased immunoglobulin (Ig)G. The diseases are usually self limited, but involvement over bones may lead to marked functional impairment. Those on the face may be associated with underlying abnormalities and, occasionally, seizures. Treatment regimens are difficult to assess as there is no chemical marker, but penicillamine has gained wide use. It has numerous side effects and patients should be carefully monitored, particularly with regard to renal function. Other modalities have been used with variable success, but series of patients are small. Many medications produce skin softening in patients with progressive systemic sclerosis. The pathogenesis of skin sclerosis involves complicated interactions between vascular responses, lymphokines, and connective tissue proliferation. The etiology is completely unknown, despite a few cases associated with increased Borrelia titers.
A 46-year-old woman who presented with progressive facial hemiatrophy (PFH) following localized scleroderma is described. The patient had a markedly deformed and depressed plaque surrounded by erythema on the right cheek. She also showed linear scleroderma with hair loss on the occipital area and morphea lesions on the neck and shoulder. Histological findings of the facial and other atrophic lesions were consistent with localized scleroderma. Therefore, the PFH and localized scleroderma may have had the same pathological background in this case.
Prevalence, levels, and immunoglobulin classes of anti-single-stranded DNA antibodies were determined by an enzyme-linked immunosorbent assay in 52 patients with localized scleroderma (33 with morphea, four with generalized morphea, and 15 with linear scleroderma), in 60 healthy controls, and, for comparison, in 31 patients with systemic lupus erythematosus. Localized scleroderma revealed a significant prevalence of anti-single-stranded DNA antibodies, mainly characterized by high levels and IgM and IgA isotypes. Comparison of antibody characteristics in different clinical forms of localized scleroderma showed some significant differences (levels and immunoglobulin isotypes). Comparison with systemic lupus erythematosus showed that frequency, high levels, and IgG isotype of anti-single-stranded DNA antibodies significantly prevailed in systemic lupus erythematosus, while the IgM isotype significantly prevailed in localized scleroderma. However, generalized morphea and linear scleroderma did not significantly differ from systemic lupus erythematosus as regards antibody frequency and prevalence of high antibody levels.
Localized scleroderma involves primarily skin but also muscle, bone and synovium. Further associations and transitional forms have been reported. We report here two cases showing associations between localized scleroderma and vasculitis, mononeuritis multiplex, juvenile chronic arthritis and hemiatrophy. In particular our cases both possess antibodies to double-stranded DNA, a finding not previously reported.
Two patients had a distinctive variant of localized scleroderma. Both have a history of sclerodermatous changes of the skin over the face developing relatively late in life and accompanied by hair loss, cutaneous calcification, and prominent beaking of the nose. A striking lack of systemic involvement also was noted.
OBJECTIVES AND DESIGN: In vivo capillaroscopic examination was performed on patients with localized scleroderma to determine whether nailfold capillary abnormalities seen in systemic scleroderma (systemic sclerosis) were also present in the localized form. Twenty-seven patients (24 women, three men) were examined by this technique. RESULTS: Only two patients exhibited scleroderma-type nailfold capillary abnormalities similar to those seen in systemic sclerosis. Both patients also suffered from Raynaud's phenomenon and showed evidence of coexisting systemic sclerosis, one on first examination, the other 1.5 years later. Our results are compared with earlier studies reporting such rare coexistence of the two forms of scleroderma. Earlier capillaroscopic work in this disorder is also reviewed. CONCLUSIONS: These results suggest that the presence, in a patient with localized scleroderma, of nailfold capillary abnormalities similar to those seen in systemic sclerosis should alert the physician to a possible association with systemic sclerosis.
Ultrasonographic methods have recently provided us with the means for objective and non-invasive monitoring of the dynamics of chronic skin diseases. We examined 34 patients with localized scleroderma (morphea) using a 20-MHz B-mode ultrasound scanner (DUB 20, Taberna pro Medicum, Lüneburg). In patients with plaque-type and linear band-type localized scleroderma intraindividual comparison of sclerotic skin with corresponding areas of healthy skin showed thickening of the corium. The increase in corium thickness was between 2% and 251%. The extent of the difference in corium thickness between sclerotic and healthy skin depended on the location-originally thin skin showed a greater degree of sclerosis. We also frequently found enhanced reflexes in the lower corium and hyperechoic, widened bands of connective tissue traversing the subcutaneous fatty tissue from the corium-subcutis border in the direction of the muscle fascia. 20 patients were examined several times in the course of one year. In nine patients we found ultrasonographic evidence of regression (decrease in thickness 26%) and in nine the ultrasound examination showed progression (increase in thickness 28%). 20-MHz B-mode ultrasound imaging is a suitable non-invasive method for monitoring the course and treatment of localized scleroderma. Its routine use is strongly recommended.
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The unique coincidence of five dermatological disorders, which occurred in a 39-year-old patient, is discussed. The clinical and laboratory examination did not reveal a common underlying cause. It is hoped this report will stimulate the recognition of other cases and thus aid in determining whether the coincidence of these disorders is a true association of diseases with a common underlying factor or a rare abnormality.
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