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Results for “Scleroderma, Localized”

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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↗
PubMed1994

Treatment of linear localized scleroderma with the anti-allergic drug, tranilast.

A 14-year-old boy with linear localized scleroderma had a dramatic improvement in contractures after treatment with N-(3',4'-dimethoxycinnamoyl) anthranilic acid (tranilast, Rizaben). The observation that this anti-allergic drug was effective in localized scleroderma lends further support to the concept that mast cells play a role in increased collagen synthesis in this disease.

Adolescent↗
PubMed1993

Squamous cell carcinoma in localized scleroderma following immunosuppressive therapy with azathioprine.

A 40-year-old man presented with an ulcerated tumour in a fibrotic plaque on the dorsum of his left foot. Due to severe localized scleroderma, the patient had been treated with azathioprine 10 years earlier. Histopathology of the excised tumour revealed an anaplastic squamous cell carcinoma within a scar of localized scleroderma. The case demonstrates that not only patients with tense scar tissue following burning, congelation, chronic radiodermatitis, lupus vulgaris or lupus erythematosus but also patients who have had localized scleroderma may run a greater risk of developing squamous cell carcinoma. Immunosuppressive therapy has to be discussed as an additional risk factor in our patient. Therefore, narrow clinical follow-up was recommended for early detection of relapse.

Adult↗
PubMed1991

20-MHz B-mode ultrasound in monitoring the course of localized scleroderma (morphea).

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.

Adolescent↗
PubMed1990

Localized scleroderma--response to 1,25-dihydroxyvitamin D3.

1,25-Dihydroxyvitamin D3 [1,25(OH)2 D3] may be an immunomodulatory drug which could have a role in controlling collagen deposition, and inducing reversal of fibrosis in some tissues. These observations prompted a study of the possible use of this hormone for the treatment of scleroderma. A 35-year-old woman, who had been suffering from localized scleroderma for 2 years, was given oral 1,25(OH)2 D3 for 6 months. The effects of the treatment were evaluated using clinical and physical measurements (skin thickness, extensibility properties of the skin). The evolution of the patient's condition during the 6-month therapy suggests that 1,25(OH)2 D3 is beneficial in localized scleroderma. The mechanisms of action are discussed in relation to the literature, which suggests both immunoregulatory and inhibitory effects on fibroblast growth.

Adult↗
PubMed1984

Collagen specific amino acids in skin in localized scleroderma.

Hydroxyproline, hydroxylysine and proline were determined on skin from 18 patients with localized scleroderma (10 with localized morphoea plaque and 8 with generalized morphoea). Three skin biopsies (4mm punch) were obtained from each patient: One from the center of a sclerotic plaque, one from the perilesional area, and one (control) from unaffected skin of the same region. Clinically, the sclerosis was more pronounced (p less than 0.01) in localized morphoea plaque as compared to generalized morphoea. Patients with localized morphoea plaque had an increased concentration of hydroxylysine (p less than 0.01) and an increased ratio of hydroxylysine to hydroxyproline (p less than 0.01) in the plaques. Hydroxylysine concentration was not changed in patients with generalized morphoea. In the entire material, increased hydroxylysine concentration were related to shorter age of the plaques (p less than 0.05) and to advanced degree of sclerosis (p less than 0.05). The hydroxylysine and hydroxyproline content per mm2 skin surface, and the weight of the dried defatted biopsy cores were increased in sclerotic plaques (p less than 0.01) in localized as well as generalized morphoea. There were no changes in the hydroxyproline and proline concentrations in any of the groups. Specimens from perilesional area showed intermediate changes. The results were compared with selected cases of lichen sclerosis et atrophicus and atrophic skin diseases. The increase in hydroxylysine concentration and ratio to hydroxyproline indicate that patients with localized morphoea plaque contain an increased proportion of newly synthesized collagen in the fibrotic plaque.

Adolescent↗
PubMed2001

Localized scleroderma of the breast.

We report a 44-year-old patient with right-breast morphea. Mammography, MRI and needle biopsy were used for assessment of the case. Mammography demonstrated thickening of the skin and the subcutaneous tissue. The MRI showed replacement of the subcutaneous and breast fat by a low signal intensity, non-enhancing tissue. Skin biopsy confirmed the histological features of scleroderma.

Adult↗
PubMed1985

Clinical and serologic expression of localized scleroderma. Case report and review of the literature.

A patient is presented in whom both linear scleroderma and eosinophilic fasciitis developed. The latter is rare in childhood. A positive antinuclear antibody, a positive rheumatoid factor, and seizures also developed. In reviewing the literature, we concluded that the clinical and histologic pattern of cutaneous sclerosis may be more valuable than serologic findings in predicting both the likelihood of, and the expected sites for, systemic involvement. The literature also suggests that central nervous system (CNS) involvement is rare in most sclerosing syndromes but that linear scleroderma may be associated with CNS or spinal disease. The ipsilateral association of linear scleroderma and seizure focus in this patient, as well as the focal onset of these seizures, is unusual and suggests an association between the seizure disorder and the linear cutaneous sclerosis.

Adolescent↗
PubMed1987

Localized scleroderma (morphea) and antibody to Borrelia burgdorferi.

Enzyme-linked immunosorbent assay (ELISA) and immunofluorescence assay (IFA) were performed in 25 and 32 cases of morphea, respectively. The more sensitive and specific ELISA was positive in only 1 of 25 cases and the mean value was lower in cases of morphea than in controls. IFA showed minimally reactive titers in 6 (19%) of 32 cases. There were an additional six cases with borderline titers. These data indicate that there is no specific association between Borrelia burgdorferi infection and morphea but that patients with morphea tend to have circulating antibodies that are cross-reactive.

Antibodies, Bacterial↗