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Results for “Scleroderma, Localized”

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PubMed1982

[Necrobiosis lipoidica].

The most important morphological aspects and pathogenesis of necrobiosis lipoidica concerning histological and clinical aspects are reported. In case of diabetes we find more frequently necrobiosis lipoidica localized out of the shank than in cases of necrobiosis lipoidica without diabetes. Necrobiosis lipoidica must be differentiated from granulomatosis disciformis, localized scleroderma and atrophy of the skin of another origin. Beside normalization of diabetes and application of corticosteroids physical and surgical treatment is recommended.

Adrenal Cortex Hormones↗
PubMed2002

Phototherapy for scleroderma: biologic rationale, results, and promise.

Scleroderma is a chronic disease of connective tissue characterized by deposition of large amounts of collagen. Localized scleroderma affects only the skin, whereas systemic disease, systemic sclerosis, may affect the lungs, gastrointestinal tract, kidneys, and heart in addition to the skin. Although the various forms of localized scleroderma are not life threatening, they result in considerable morbidity owing to joint contracture, loss of flexibility, and disfigurement. Although many different treatments have been attempted, until now none has proven to be effective. Accumulating evidence indicates that UVA irradiation offers a genuine opportunity to ameliorate localized scleroderma and the cutaneous manifestations of systemic sclerosis.

Humans↗
PubMed2002

Localized linear scleroderma with cutaneous calcinosis.

A 38-year-old woman developed sclerotic and atrophic changes of the left femur in the winter of 1976. In 1980, she was referred to our dermatology clinic and was diagnosed with localized linear scleroderma from the results of the physical examinations and the histological findings. Although several local and systemic treatments were employed over the following 10 years, the sclerotic lesion did not show any remarkable improvement. In 1991, several hard and white papules appeared in the lesion, and a biopsy specimen of these white papules revealed calcinosis.

Adult↗
PubMed2001

Different pattern of collagen cross-links in two sclerotic skin diseases: lipodermatosclerosis and circumscribed scleroderma.

Changes in the process of cross-linking of collagen molecules are associated with defects in the biomechanical stability of the extracellular matrix. Fibrosis of skin is characterized by an increase in pyridinolines, which are hydroxylysine aldehyde derived cross-links usually absent in healthy skin. In this study, we analyzed cross-links in lipodermatosclerosis and localized scleroderma to address the question whether all the mature cross-links currently characterized are increased in fibrosis in addition to the increase in pyridinolines. As psoralen plus ultraviolet A treatment leads to clinical improvement of fibrotic plaques in localized scleroderma we analyzed the cross-link content in lesional skin after bath psoralen plus ultraviolet A therapy. In skin from patients with localized scleroderma an increase in the total number of mature cross-links was found to be due to an increase in both pyridinolines and dehydro-histidinohydroxymerodesmosine. The concentration of histidinohydroxylysinonorleucine was unchanged. By contrast, the total number of mature cross-links was decreased in lipodermatosclerosis. This decrease was caused by a decrease of lysine aldehyde derived cross-links (dehydro-histidinohydroxymerodesmosine and histidinohydroxylysinonorleucine), whereas the concentration of pyridinolines increased. A decrease in the content of pyridinolines after bath psoralen plus ultraviolet A treatment was found in six out of nine patients with localized scleroderma, which might reflect a remodeling of the extracellular matrix. Our data provide evidence that sclerosis of skin is associated with either an increase in the number of cross-links per molecule of collagen or a change in the molecular nature of the cross-links formed.

Amino Acids↗
PubMed1989

Eosinophilic fasciitis.

The fascia had received little attention until Shulman's delineation of EF. Evidence is now accumulating that in addition to EF and scleroderma, significant fascial inflammation may be seen in polymyositis, dermatomyositis, eosinophilic polymyositis, systemic lupus erythematosus, and mixed connective tissue disease. It is still unclear whether EF represents a variant of scleroderma; however, it is becoming increasingly recognized that scleroderma shares many features in common with EF including eosinophilia, hypergammaglobulinemia, positive ANA and rheumatoid factor, and an association with hematologic disease. The rarity of Raynaud's phenomenon and significant visceral changes help distinguish EF from systemic scleroderma. In this regard, however, EF more closely resembles the localized scleroderma syndromes, especially morphea profunda and pansclerotic morphea. Biopsy in EF, systemic scleroderma, and localized scleroderma will show comparable changes, the essential difference being the levels at which they occur.

Eosinophilia↗
PubMed1992

[Facial hemiatrophy, homolateral cervical linear scleroderma and thyroid disease].

A case of facial hemiatrophy and homolateral cervical band of scleroderma, complicated by hypothyroidism is reported. This case raises two problems: one is the problem of distinction between Romberg's disease and facial hemiatrophy due to a genuine localized scleroderma; the other concerns the relationship between localized scleroderma and dysthyroidism. The generalized scleroderma-dysthyroidism association has now been recognized, but the coexistence of thyroid disease and localized scleroderma has not yet been reported. Several pathogenetic hypotheses on this association are discussed.

Facial Dermatoses↗
PubMed1990

Cyclosporin in localized and systemic scleroderma--a clinical study.

Four patients with systemic scleroderma and 1 patient with localized scleroderma were treated with ciclosporin (CS), given in daily doses between 2.2 and 5.6 mg/kg body weight for 3-26 months. Under this medication clinical improvement was observed in 4 patients with partial regression of cutaneous sclerosis and inflammation, healing of fingertip ulcerations or leg ulcers and improvement of articular mobility. However, in 1 patient with rapidly advancing systemic scleroderma a short-term therapy with CS in low doses (2-3 mg/kg body weight) resulted in arterial hypertension and renal dysfunction. Therefore careful selection of patients and close-meshed controls are indicated when CS is considered as anti-inflammatory treatment in scleroderma.

Acute Kidney Injury↗
PubMed1977

[Idiopathic and sclerodermic facial hemiatrophy with generalized myopathy. Clinical, electromyographic and histologic examinations of six patients].

Six patients with facial hemiatrophy (H.f) were thoroughly examined by clinical and laboratory investigations. Two were found to have idiopathic and four facial hemiatrophy due to different types of localized scleroderma. In all cases a generalized myopathy was present, demonstrated by electromyographical, histological, and biochemical means. In none of these cases a hereditary or neurological cause for the facial hemiatrophy was found. However, in two cases autoantibodies against nuclei and muscle were repeatedly obtained. No prolongation of sensory or pain chronaxy occurred in either the patients with sclerodermal or idiopathic facial hemiatrophy. These observations suggest that facial hemiatrophy can originate (a) in a localized scleroderma in the involved part of the face, (b) in an ipsilateral "sclérodermie en coup de sabre", or (c) in the coexistence of both. There is a close pathological and physiological correlation between idiopathic and sclerodermal facial hemiatrophy. In both forms of facial hemiatrophy the disease involves skeletal muscle tissue systematically. This myopathy is similar to that of progressive scleroderma, far exceeding the limited muscular involvement of localized scleroderma. Sclerodermal facial hemiatrophy can be associated with autoimmune phenomena.

Adipose Tissue↗
PubMed1981

Myopathy associated with sclerodermal facial hemiatrophy.

A patient who had linear scleroderma associated with ipsilateral hemiatrophy of the tongue and subsequent facial hemiatrophy was studied. Biopsy specimens of the plaque of scleroderma showed the skin changes of scleroderma as well as fascial and muscle changes. The fascia had an impressive plasma cell fasciitis with numerous plasma cells and scattered lymphohistiocytic cells. Histochemical study of the temporalis muscle underlying the plaque of circumscribed scleroderma disclosed severe localized atrophy of type 1 and type 2 fibers similar to the pathologic findings previously described in a patient with localized scleroderma.

Adult↗
PubMedDate not supplied

Acrokeratoelastoidosis associated with nodular scleroderma.

We report a case of acrokeratoelastoidosis associated with nodular scleroderma, a variant of localized scleroderma. The patient showed no clinical and laboratory signs of systemic scleroderma. Because there is a paper describing the association of acrokeratoelastoidosis with systemic scleroderma, coexistence of acrokeratoelastoidosis and nodular scleroderma in the patient suggests that acrokeratoelastoidosis is related to localized nodular scleroderma as well as systemic scleroderma.

Abdomen↗
PubMed1994

Plasma endothelin and the aminoterminal propeptide of type III procollagen (PIIINP) in systemic sclerosis.

Forty-four patients with systemic sclerosis and 3 patients with localized scleroderma were investigated for plasma endothelin and aminoterminal propeptide of type III procollagen (PIIINP). Although there was an overlap between plasma levels of endothelin in patients with systemic sclerosis and healthy controls, the mean value of the patients was significantly higher than in controls. Plasma endothelin was normal in all 3 patients with localized scleroderma. The highest levels of plasma endothelin were found in patients with type II and III systemic sclerosis with the largest cutaneous involvement, and in patients with the scleroderma-specific antibodies Scl-70 and anticentromere antibodies. Extremely high values were found in a patient who experienced a renal crisis and in a patient who had her lower leg amputated due to severe vasculitis. A positive correlation was found between plasma endothelin and serum PIIINP. This, together with the fact that in systemic sclerosis the vascular lesions are the earliest, would seem to support the theory that endothelial cell damage could lead to increased secretion of endothelin and subsequent fibrosis in this disease.

Aged↗
PubMed1987

Antinuclear and anti-single-stranded DNA antibodies in morphea and generalized morphea.

The clinical features of localized scleroderma have allowed investigators to distinguish three morphologic variants: morphea, generalized morphea, and linear scleroderma. The latter has been reported to have a higher frequency of antinuclear antibodies and has been associated with antibodies to single-stranded DNA (ssDNA). In this study we determined the frequency of antinuclear antibodies and anti-ssDNA antibodies in 22 patients with morphea or generalized morphea. None had Raynaud's phenomenon or evidence of a systemic connective-tissue disease. Antinuclear antibodies were present in 18% of patients when serum samples were tested on mouse kidney substrate but were found in 50% of HEp-2 cells. The serum samples contained anti-ssDNA antibodies in 59% of the patients, with the highest levels of ssDNA binding observed in patients with generalized morphea. The frequency of antibodies to ssDNA was higher in patients with clinical evidence of active compared with inactive disease. Discordance in immune reactivity indicates that at least three distinctive serum autoantibodies exist in morphea and generalized morphea: anti-ssDNA antibodies and antinuclear antibodies with either homogeneous or speckled immunofluorescence patterns. These findings are similar to those recently described in linear scleroderma and suggest that comparable serum autoantibody abnormalities are present in all the variants of localized scleroderma.

Adolescent↗