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Results for “Scleroderma, Localized”

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At least 559 records · Page 31Linked to original sources
PubMed1997

Antiendothelial cell antibodies: useful markers of systemic sclerosis.

BACKGROUND: Systemic sclerosis (SS) encompasses a wide spectrum of clinical presentations. Antiendothelial cell antibodies (AECA) in patients with primary Raynaud's phenomenon (PRP), limited SS (lSSc), or diffuse SS (dSSc) may help to determine the long-term prognosis of the disease. METHODS: Twenty-seven normal controls, 13 patients with PRP, 36 with lSSc, and 31 with dSSc were included in the study. Sera were examined for the presence of AECA, using a cellular enzyme-linked immunosorbent assay (ELISA). Angiotensin-converting enzyme (ACE) activity, plasma von Willebrand factor antigen (vWfAg), and thrombomodulin (Tm) concentrations were also evaluated. The medical records of 50 of the lSSc and dSSc patients were reviewed and the organ system involvement noted. RESULTS: Antiendothelial cell antibodies were present in 3 patients with PRP, 16 patients with lSSc, and 26 patients with dSSc. These autoantibodies were mainly of the IgG isotype. There was no difference in ACE activity between patients and controls. In contrast, vWfAg and Tm concentrations were higher in patients with PRP relative to controls, and higher in patients with lSSc compared with those with PRP. The presence of AECA was associated with digital scars and ulcers (P < 0.004 and P < 0.003, respectively), severe RP (P < 0.01), grade 3 tortuosity of vessels (P < 0.0004), and lung involvement (P < 0.02). CONCLUSION: The significant trend for AECA to increase with disease severity across the three groups of patients studies suggests that the AECA test can identify subsets of SSc with differing prognoses.

Adolescent↗
PubMed1998

Collagen vascular diseases.

Collagen vascular diseases are multisystem disorders that frequently affect the skin. At times, cutaneous disease is the initial manifestation. This article focuses on lupus erythematosus, dermatomyositis, and sclerodermoid syndromes.

Collagen Diseases↗
PubMed1998

Cutaneous manifestations of systemic diseases.

Many aspects of dermatologic diagnosis are either of importance or interest to the nondermatologist, and there are many excellent textbooks available for guidance. This article focuses on four categories of conditions that are the source of frequent queries from the primary care setting: (1) common skin diseases that frequently mimic systemic illness, (2) common skin diseases that have important systemic associations, (3) common systemic diseases that have prominent cutaneous findings, and (4) the seldom seen but frequently raised concerns regarding cutaneous signs of internal malignancy.

Cellulitis↗
PubMed1986

Scleroderma in childhood.

Scleroderma is a rare disease in children. Heterogeneous in its many forms, it ranges from circumscribed and self-limited pigmentary disorders to disabling and disfiguring involvement of an extremity. It also may include diffuse skin disease, multiple internal organ derangements, and a rapidly fatal outcome. Outcome varies widely and is related to the location and extent of cutaneous involvement, evaluation of disfigurement or impairment, and which treatment resources are available.

Child↗
PubMed1995

Scleroderma in children.

Childhood scleroderma may present in a variety of clinical forms that differ in clinical presentation, extracutaneous features, clinical course, and outcome. All include hardening of the skin as a major feature. This article reviews these various entities, focusing on primarily the clinical features. In addition, current concepts regarding pathogenesis and treatment are discussed.

Adolescent↗
PubMed1991

Fibrin and fibrinogen-related antigens in systemic sclerosis (scleroderma)

Abnormalities in fibrin deposition are implicated in the pathogenesis of vascular occlusion in systemic sclerosis. We have used a technique that involves electrophoresis and densitometric analysis of captured fibrin- and fibrinogen-related antigens to measure the concentration of the individual fibrin and fibrinogen degradation products in 13 patients with systemic sclerosis and in 15 healthy control subjects. As a group, patients with systemic sclerosis had markedly elevated levels of total fibrin-related antigen (p = 0.0007) and D-dimer (p = 0.0004), the terminal degradation product of cross-linked fibrin. The levels of fibrin monomer, an intermediate product in the conversion of fibrinogen to cross-linked fibrin, and of D-monomer, a terminal breakdown fragment of fibrinogen and fibrin monomer, were also elevated (p less than 0.005). We conclude that patients with systemic sclerosis have evidence of enhanced fibrin formation and degradation.

Adult↗
PubMed1987

Scleroderma.

After a review of some pathogenetic and pathologic aspects of scleroderma, the experimental effects of a group of agents that can inhibit the formation of connective tissue, especially the biosynthesis of collagen, are mentioned. These substances were transferred to clinical therapy of scleroderma. Regular determinations of disease activity and guidance of therapy with quantitative and semiquantitative physical and biochemical technics are of utmost importance because treatment without guidance allows for no disclosure of therapeutic failure or recurrence of the disease in due time for readjustment of the treatment.

Adolescent↗