Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Scleroderma, Localized”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 73 records · Page 4Linked to original sources
PubMed1992

Progressive facial hemiatrophy with localized scleroderma.

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.

Facial Hemiatrophy↗
PubMed1995

Classification of morphea (localized scleroderma)

OBJECTIVE: To classify and describe morphea (localized scleroderma). DESIGN: A review of morphea and its subtypes is presented. RESULTS: The current classification of morphea is incomplete and confusing. As knowledge of the spectrum of disease continues to evolve, the controversy and confusing nature of its multiple subtypes present a challenge for the physician who encounters a patient with this condition. Thus, we propose that morphea be classified into the following five groups: plaque, generalized, bullous, linear, and deep. This classification, based on clinical morphologic findings, will simplify the diagnostic and therapeutic approach. CONCLUSION: Morphea represents a wide variety of clinical entities that seen to be on the opposite end of the scleroderma spectrum from systemic sclerosis. The cutaneous lesions eventually evolve from a sclerotic stage to a nonindurated stage, and residual hypopigmentation or hyperpigmentation follows. The histologic pattern in patients with morphea is similar to that in patients with progressive systemic sclerosis. Although treatment is nonstandardized, hydroxychloroquine sulfate may be beneficial.

Humans↗
PubMedDate not supplied

Localized scleroderma and idiopathic thrombocytopenia.

Two patients with localized scleroderma who developed thrombocytopenic purpura are reported. In both patients the thrombocytopenia improved after prednisone therapy, and has not recurred in 5 and 8 yr of subsequent observation without steroids. Both patients had a positive antinuclear antibody (ANA) test and 1 had a positive assay for immune complexes by the Clq solid phase radioimmunoassay. Non-immune causes for the lowered platelets were not found. This possible association may add a new, potentially life-threatening dimension to localized scleroderma.

Adolescent↗
PubMed1995

Autoantibodies to the heat-shock protein hsp73 in localized scleroderma.

We determined the presence of antibodies to the heat-shock protein hsp73 (anti-hsp73) in 57 serum samples from patients with localized scleroderma using an enzyme-linked immunosorbent assay (ELISA). In addition, 30 samples from healthy individuals, 30 from patients with systemic lupus erythematosus (SLE) and 32 from patients with systemic sclerosis were assessed. IgG and/or IgM anti-hsp73 antibodies were detected in 33% (19/57) of the patients with localized scleroderma. Among the three subtypes of localized scleroderma, generalized morphoea showed the highest incidence of anti-hsp73 antibodies (40%, 6/15). IgG and/or IgM anti-hsp73 antibodies were also detected in 9/30 samples (30%) from patients with SLE and in 13/32 samples (41%) from patients with systemic sclerosis, while the samples from the healthy controls were all negative for anti-hsp73. By immunoblotting, specific binding of antibodies to hsp73 was confirmed with representative serum samples that were positive for anti-hsp73 in the ELISA. Our findings indicate that the presence of anti-hsp73 is an additional immunological abnormality in localized scleroderma.

Adolescent↗
PubMed1996

Elevated soluble CD23 levels in the sera from patients with localized scleroderma.

Soluble CD23 (sCD23) is closely related to B-cell activation and elevated serum levels of sCD23 have been reported in several autoimmune disorders. This study investigated the serum levels of sCD23 and determined the correlation of sCD23 with other immunologic abnormalities and clinical features in localized scleroderma. We examined 49 serum samples by an enzyme-linked immunosorbent assay in the following three subgroups: 15 patients with generalized morphoea, 22 with linear scleroderma, and 12 with morphoea. The serum levels of sCD23 were significantly elevated in patients with localized scleroderma, compared with those in healthy individuals. Of the three subgroups of localized scleroderma, patients with generalized morphoea had the highest levels of serum sCD23. The frequency of IgM antihistone antibody (AHA) and IgM rheumatoid factor (RF), the number of linear lesions, and the frequency of muscle involvement were significantly higher in patients with elevated sCD23 levels than in those with normal levels of sCD23. A significant correlation between the serum sCD23 level and the number of involved areas of the body was observed. Our data suggest that the activation of virgin B cells, which is reflected by elevated sCD23 levels, is closely associated with the production of IgM autoantibodies in localized scleroderma and furthermore that the serum levels of sCD23 are a new serological indicator of the severity of localized scleroderma.

Adolescent↗
PubMed1984

Treatment of localized scleroderma and lichen sclerosus with etretinate.

Eight patients with lichen sclerosus and 8 patients with localized scleroderma were treated with etretinate (Tigason) at doses of 0.5 mg/kg body weight for between 3 and 12 months: 2 of 3 patients with juvenile type lichen sclerosus cleared, whereas the lesions of 5 patients with balanitis xerotica obliterans, kraurosis vulvae and extragenital lichen sclerosus were unchanged or even progredient. 2 of the patients with localized scleroderma cleared with slight atrophy. The lesions of 5 others failed to resolve but progression was halted under continued therapy; after drug withdrawal, the lesions resumed progression in 2 patients. We conclude that only moderate therapeutic effects can be achieved with etretinate in both lichen sclerosus and localized scleroderma.

Adult↗
PubMed1994

Localized scleroderma in childhood: a report of 30 cases.

Localized scleroderma (LS), a rare disease that occurs primarily in the pediatric age group, differs from systemic sclerosis (SSc) in that it is usually limited to the skin and subcutaneous tissue and is only rarely associated with systemic manifestations. The authors' experience with pediatric LS seen in 30 patients at a tertiary care center was reviewed: 26 had linear scleroderma, 19 on an extremity and 7 on the face; 3 had morphea; and 1 had generalized morphea. Antinuclear antibodies were present in 76% and rheumatoid factor in 39%. Five of 19 patients with linear scleroderma that involved an extremity had growth failure in that limb, and 1 required surgery. Sclerodermatous involvement over a joint resulted in limited range of movement in 6 patients, and 1 required surgery. One of the 30 patients developed SSc and polymyositis. There was difficulty in evaluating disease activity and hence in evaluating treatment. This experience with a large patient population suggests that LS, although usually a self-limiting disease, can result in significant morbidity.

Adolescent↗
PubMed1991

Prevalence and characteristics of anti-single-stranded DNA antibodies in localized scleroderma. Comparison with systemic lupus erythematosus.

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.

Adult↗
PubMed1992

Localized scleroderma and hemiatrophy in association with antibodies to double-stranded DNA.

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.

Adolescent↗
PubMed1994

Serum levels of soluble interleukin-2 receptor. A marker of disease activity in localized scleroderma.

OBJECTIVE: To determine whether circulating serum levels of soluble interleukin-2 receptor (sIL-2R) are elevated in patients with localized scleroderma, and if levels of sIL-2R can differentiate between active and inactive disease. METHODS: Seventeen patients with localized scleroderma were categorized by overall physician assessment into active, inactive, and indeterminate groups, according to disease activity. Serum sIL-2R levels were analyzed and correlated with disease activity. RESULTS: The mean sIL-2R level was significantly higher (P = 0.005) in those with active disease (1,675 +/- 823 units/ml) than in those with inactive disease (722 +/- 218 units/ml). CONCLUSION: Serum sIL-2R levels are elevated in patients with localized scleroderma. When present, elevated sIL-2R levels appear to be able to differentiate active from inactive disease. This fact also suggests cell-mediated immune activation in this condition. Further serial studies are required to assess the value and sensitivity of sIL-2R levels in measuring changes in disease activity.

Adolescent↗
PubMed1979

Localized scleroderma with cutaneous calcinosis. A distinctive variant.

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.

Aged↗
PubMed1985

Glycosaminoglycans in localized scleroderma (morphoea).

The composition of glycosaminoglycans (GAGs) was analyzed in skin samples of eight patients suffering from localized scleroderma, i.e., three having generalized morphoea and five localized morphoea plaques. From each patients, biopsies were obtained from sclerotic and perilesional areas, and from clinically uninvolved skin of the same region. In the perilesional areas hyaluronic acid concentration was increased (p less than 0.05), while it was decreased (p less than 0.01) in sclerotic areas. Dermatan sulfate concentration was increased in the perilesional (p less than 0.05) as well as in the sclerotic (p less than 0.01) areas. Chondroitin 4/6 sulfate was increased in the sclerotic areas (p less than 0.05). Heparan sulfate showed no changes. No major differences were found in the total concentrations of uronic acid and hexosamine. This study demonstrates that changes in GAG composition in localized scleroderma follow previously described sequences of events of inflammation and fibrosis of connective tissue.

Adolescent↗
PubMed1994

Serum concentration of procollagen type I carboxyterminal propeptide in localized scleroderma.

BACKGROUND AND DESIGN: Recently, we reported that the serum procollagen type I carboxyterminal propeptide (P1CP) level of patients with systemic sclerosis was elevated and correlated with disease severity. In this study, the serum level of P1CP was measured using the enzyme-linked immunosorbent assay in 39 patients with localized scleroderma and in 30 control subjects. RESULTS: The mean P1CP level in the patients was significantly higher than that in the normal control subjects. In 30% of the patients with localized scleroderma, the serum P1CP level was considered to be elevated (> 305 ng/mL; ie, 2 SDs above the mean control value). The mean serum P1CP level in the patients with generalized morphea was significantly higher than in the patients with morphea or linear scleroderma. In addition, the serum P1CP level in the patients with localized scleroderma was correlated with the number of sclerotic lesions, and it was negatively correlated with the duration of the disease. Anti-single-stranded DNA antibody and antihistone antibody were detected significantly more frequently in the patients with elevated P1CP than in the patients with normal P1CP. CONCLUSION: These findings suggest that the serum P1CP level is a useful indicator of disease severity in patients with localized scleroderma, as it has been found to be in those with systemic sclerosis.

Adolescent↗
PubMed1995

Treatment of severe localized scleroderma by plasmapheresis--report of three cases.

We report three patients with severe, localized scleroderma, and with elevated titres of antinuclear antibodies, who were treated by plasmapheresis in combination with systemic steroid therapy. The therapeutic effectiveness of plasmapheresis was assessed on the basis of improvement in cutaneous and joint lesions. In all cases, significant improvement occurred after 2 months of therapy. Thus, in addition to treating systemic sclerosis, plasmapheresis can also be recommended for treatment of severe cases of localized scleroderma with elevated titres of antinuclear antibodies and antibodies to ss-DNA.

Adult↗
PubMed1990

Progressive inflammatory lesions of the brain parenchyma in localized scleroderma of the head.

A patient with localized scleroderma of the head, uveitis, and Raynaud's phenomenon presented with generalized seizures, spastic hemiparesis, and local IgG production in the cerebrospinal fluid. Magnetic resonance imaging revealed progressive cortical and subcortical brain parenchymal lesions mainly adjacent to the cutaneous and bony lesions and probably of inflammatory origin.

Adult↗
PubMed1984

Collagen metabolites in urine in localized scleroderma (morphoea).

Hydroxyproline (Hyp), hydroxylysine (Hyl), and proline (Pro) were assayed in the urine from 28 patients with localized scleroderma (13 with localized morphoea plaques, 13 with generalized morphoea, and two with scleroderma en coup de sabre), as well as 17 control persons. The levels of Hyp, Hyl and Pro were elevated in patients with localized morphoea plaques as compared to the controls indicating major changes of the collagen metabolism in this type of localized scleroderma.

Adolescent↗
PubMed1992

Capillary abnormalities, Raynaud's phenomenon, and systemic sclerosis in patients with localized scleroderma.

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.

Adolescent↗