Cases reported "Scleroderma, Localized"

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1/9. Post-stripping sclerodermiform dermatitis.

    BACKGROUND: Cutaneous sclerosis, a process that results in hardening of the skin, is the hallmark of scleroderma and sclerodermoid disorders. Cutaneous sclerosis is usually classified as secondary or primary, depending on the presence or absence of underlying diseases. Primary cutaneous sclerosis is a feature of idiopathic inflammatory processes that are often associated with autoimmune disorders, whereas secondary cutaneous sclerosis arises in the context of many pathological processes of varying causes, including chronic graft-vs-host disease, defined metabolic or genetic disorders, and exposure to certain infectious organisms, drugs, or chemicals. OBSERVATIONS: Three patients had localized cutaneous sclerosis overlying the site of a surgically removed (stripped) great saphenous vein. In all 3 patients, lesions were clinically characterized by multiple hypopigmented and indurated plaques distributed linearly along the path of the preexisting vein. Extensive history, physical examination, and diagnostic tests did not reveal known predisposing factors for cutaneous sclerosis. CONCLUSIONS: Although the observed association of sclerodermiform dermatitis and venous stripping in these 3 patients does not imply a causal relationship, the absence of other identifiable predisposing factors and the striking linear distribution of the cutaneous lesions along the path of the preexisting vein are suggestive. This poststripping sclerodermiform dermatitis may be a rare late complication of saphenous vein stripping.
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2/9. Linear scleroderma: a Neuman nursing perspective.

    Although quite a rare pediatric disease, linear scleroderma (LS), a chronic disease, may leave scarring and may have devastating effects on a child and family. This article provides an overview of the disease, presents a case history of a boy with LS, and discusses how nurses and other health-team members assisted the child and family in managing the illness. The description of the nursing care is based on the Neuman systems model and on the nursing diagnoses of fear, disturbed self-concept, and impaired physical mobility.
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keywords = physical
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3/9. Physical injury as a provoking factor in three patients with scleroderma.

    A 51-year-old female developed linear-like scleroderma in the left thigh following a linear wound caused by a car accident. 27 years later she also developed a typical diffuse cutaneous systemic sclerosis with extensive skin involvement and bibasilar pulmonary fibrosis. The second case is a 39-year-old female who had a history of Raynaud's phenomenon since early childhood. She developed a morphea following a burning injury of the left thigh. 17 years later she also developed a typical limited cutaneous systemic sclerosis with sclerodactyly, skin ulcers and subcutaneous calcinosis. The third case is a 43-year-old female who developed a typical morphea of the right elbow around the site of a previous local corticosteroid injection. The two remarkable points of these 3 cases are the possible role of physical injury in the provocation of localized scleroderma and in the first 2 cases the unusual later development of a systemic form of scleroderma.
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keywords = physical
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4/9. 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.
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5/9. ketotifen--a therapeutic agent of eosinophilic fasciitis?

    A 30-year-old male with eosinophilic fasciitis (EF) associated with morphoea and vitiligo is described. The patient showed a partial response to prednisone, but did not respond to hydroxychloroquine, D-penicillamine and cimetidine. In the light of reports of increased plasma histamine levels, and a putative role of mast cells in the pathogenesis of EF, ketotifen (a mast cell stabilizer) was prescribed. This non-toxic drug has allowed the patient to continue hard physical labour without further relapses and without corticosteroid therapy.
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keywords = physical
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6/9. Cutaneous hypermelanosis and intramelanotic lipid droplets.

    Intramelanocytic lipid droplets have been observed in 12 patients with different types of hypermelanosis of the skin. These include drug-induced hypermelanosis, hypermelanosis secondary to use of physical agents, hypermelanotic morphea, generalized scleroderma with diffuse hypermelanosis, a hyperkeratotic tumor in xeroderma pigmentosum, aberrant mongolian spot, and methoxsalen-ultraviolet-A-induced hypermelanosis. Lipid storage was selectively observed in melanocytes. Most of the melanocytes with lipid vacuoles showed ultrastructural signs of hyperactive melanosome synthesis and certain cytoplasmic abnormalities such as melanosomal autophagic vacuole formation and mitochondrial alterations. The importance and the origin of the lipid droplets remain unclear.
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keywords = physical
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7/9. Trauma-induced isomorphic lesions in morphea--a brief case report.

    We describe a case of morphea which presented further typical lesions of the disease at the sites of mechanical trauma. It can be suggested that cutaneous lesions of morphea may be locally developed due to physical stimuli as an isomorphic response in patients or subclinical cases of the disease.
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keywords = physical
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8/9. Focal scleroderma in children: an orthopaedic perspective.

    Focal scleroderma is a connective-tissue disorder manifested by fibrosis of the skin and subcutaneous tissues. Consequently, it may be associated with joint contractures, extremity deformity, and impairment of extremity function. It has a variable clinical course, with both remissions and recurrences. In resistant cases of extremity involvement, treatment considerations should include physical and occupational therapy, medical treatment, bracing, serial casting, and surgical intervention. We reviewed seven patients treated between 1960 and 1990 with significant joint contractures secondary to focal scleroderma. Serial casting was found to be useful for contractures about the wrist, knee, and ankle. Soft-tissue release was an effective treatment for contractures at the wrist, hip, and ankle. knee disarticulation was performed on one patient with a severe knee-flexion contracture. Epiphysiodesis and femoral shortening osteotomy were effective treatments for leg-length equalization in a case of hemiatrophy.
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keywords = physical
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9/9. Keratoderma with pseudo-Quincke's pulse.

    The physical examination sign known as Quincke's capillary pulse is elicited by pressing lightly on the distal edge of a fingernail. When the vasculature of the nailbed is partly blanched, Quincke's pulse appears as alternating flashes of red and white that reveal arterial pulsations. This sign is seen in many normal persons, but it is most prominent in individuals with widened pulse pressures, such as aortic insufficiency. This case report describes a patient with keratoderma and sclerodactyly whose fingernails exhibited rapid color changes, mimicking Quincke's pulse. The visible pulsations were due to sclerodactylous compression of the nailbeds, and not to any primary hemodynamic process.
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