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1/8. methotrexate-induced papular eruption in patients with rheumatic diseases: a distinctive adverse cutaneous reaction produced by methotrexate in patients with collagen vascular diseases.

    BACKGROUND: In the past few years, low doses of methotrexate have been used for treatment of patients with rheumatoid arthritis and other collagen vascular diseases, mainly as an immunosuppressive and corticosteroid-sparing drug. Several cutaneous adverse reactions have been described in association with methotrexate therapy. OBJECTIVE: We describe the clinical and the histopathologic features of distinctive cutaneous lesions that appeared in 4 patients with acute bouts of collagen vascular diseases who were receiving methotrexate therapy. methods: We clinically and histopathologically evaluated cutaneous lesions caused by methotrexate therapy in 4 patients, 2 with systemic lupus erythematosus, 1 with rheumatoid arthritis, and 1 with Sharp syndrome. RESULTS: Clinically, lesions consisted of erythematous indurated papules most commonly located on proximal areas of the extremities. Histopathologic examination of these papules showed an inflammatory infiltrate mainly composed of histiocytes interstitially arranged between collagen bundles of the dermis, intermingled with few neutrophils. In some foci of deeper reticular dermis, small rosettes composed of clusters of histiocytes surrounding a thick central collagen bundle were seen. Cutaneous lesions showed a direct chronologic relationship with methotrexate therapy, and they disappeared when the drug was tapered or withdrawn and corticosteroids were increased. CONCLUSION: patients receiving low doses of methotrexate for acute bouts of collagen vascular diseases may experience characteristic cutaneous lesions with distinctive clinical and histopathologic findings shortly after methotrexate administration. We discuss the differential diagnosis with other dermatoses showing similar histopathologic findings that have been described in patients with collagen vascular diseases.
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keywords = vascular disease
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2/8. A teenager with mixed connective tissue disease presenting with an acute coronary syndrome.

    mixed connective tissue disease (MCTD) manifests as a number of cardiovascular diseases; however, myocardial infarction secondary to coronary artery disease has not been well documented. We present a case of a teenager with MCTD and known cardiac risk factors who developed an acute coronary syndrome.
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ranking = 0.125
keywords = vascular disease
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3/8. Reactive angioendotheliomatosis in association with a well-differentiated angiosarcoma.

    A 55-year-old white female with a complex medical history including mixed connective tissue disease and peripheral vascular disease developed a group of red-purple papules on her proximal medial thigh that was followed, five months later, by the development of a large violaceous patch. She reported a history of radiation to this site (for melanoma) during her childhood. She was admitted to the hospital with a presumptive diagnosis of cellulitis, but failed to respond to antibiotics. A biopsy was performed and demonstrated a well-differentiated angiosarcoma arising in conjunction with reactive angioendotheliomatosis. Excision of the lesion was performed, and fifteen months of follow-up have shown no recurrence or metastasis.
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ranking = 0.125
keywords = vascular disease
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4/8. Condylysis in a patient with a mixed collagen vascular disease.

    Condylysis has not previously been described as a complication of a mixed collagen vascular disease. A case is presented in which apertognathia and mandibular retrognathism occurred secondary to condylysis in a 26-year-old woman with features of rheumatoid arthritis, systemic lupus erythematosus, scleroderma, and sjogren's syndrome. The disease was manifested by polyarthritis, morning stiffnes, subcutaneous nodules, and acrosclerosis. Important laboratory findings included RA slide latex negative, increased dna binding, ANA positive, ENA negative, and an abnormal parotid scan. The malocclusion secondary to condylysis was corrected by surgical procedures usually employed for the treatment of mandibular retrognathism and apertognathia on a developmental basis.
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ranking = 0.625
keywords = vascular disease
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5/8. collagen vascular disease: can behavior therapy help?

    This study examined the efficacy of a simple autogenic and biofeedback treatment package in the management of Raynaud's Phenomenon secondary to diagnosed collagen vascular disease. The patient, diagnosed as suffering from mixed connective tissue disease, had an average of 6.3 vasospastic attacks per day during a 2 week baseline period. The frequency of daily attacks dropped to 4.2 after 10 weeks and 2.5 attacks after 1 yr of training. In addition, the patient displayed a gradual improvement in the ability to maintain digital skin temperature in the presence of ambient cold stress.
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ranking = 0.625
keywords = vascular disease
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6/8. stroke and mixed connective tissue disease.

    We describe the clinical presentation and course of two girls with cerebrovascular disease and mixed connective tissue disease. One developed rapid onset hemiparesis and aphasia secondary to left internal carotid artery occlusion. She experienced a complete recovery after treatment with prednisone and cyclophosphamide. The other patient was diagnosed as having mixed connective tissue disease but had acute neurologic deterioration. She died due to an intracerebral hemorrhage. autopsy demonstrated small-vessel fibrinoid necrosis. Although cerebrovascular disease secondary to central nervous system vasculitis is a manifestation of systemic lupus erythematosus, this is the first description of cerebrovascular disease as a primary sign in mixed connective tissue disease. These cases demonstrate the range of cerebrovascular disease observed in children with mixed connective tissue disease.
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ranking = 0.5
keywords = vascular disease
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7/8. Fatal cytomegalovirus pneumonia in a patient receiving corticosteroids and methotrexate for mixed connective tissue disease.

    cytomegalovirus (CMV) pneumonia is a serious illness that may occur in severely immunocompromised patients such as those with bone marrow transplants. A strong association between this condition and immunosuppressive therapy for collagen vascular diseases has not been described. We describe a patient who had CMV pneumonia while receiving methotrexate and corticosteroids for mixed connective tissue disease.
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ranking = 0.125
keywords = vascular disease
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8/8. mixed connective tissue disease and radiation toxicity. A case report.

    BACKGROUND: Several cases of long term radiation sequelae have been reported in patients with lupus erythematosus and systemic sclerosis after breast or chest wall irradiation. To the authors' knowledge, no experience with such complications in patients with mixed connective tissue disease (MCTD) has been reported previously. methods: A case of a woman with occult breast carcinoma metastatic to the axilla and preexisting MCTD is presented. To the authors' knowledge, this is the first case report of the adverse effects of breast irradiation in a patient with MCTD. The pathophysiology of such radiation injury to specific anatomic structures and technical dosimetric considerations of the radiation therapy and radiation dose are analyzed. The relevant literature on other collagen vascular diseases with features related to MCTD is reviewed. RESULTS: A moderate dose of radiation to the breast and regional lymphatics resulted in marked early and late toxicity to skin and subcutaneous tissues. The tissue injury was similar to that observed in patients with lupus erythematosus and systemic sclerosis. The early skin reaction (moist desquamation) was related to the daily radiation dose delivered at the depth of the epidermis, and the late reaction (subcutaneous fibrosis) was related to the dose at the depth of the dermal capillaries and dermal connective tissue. CONCLUSIONS: patients with MCTD may develop exaggerated radiation reactions similar to those in patients with lupus erythematosus and systemic sclerosis. Although the incidence of such radiation reactions in patients with MCTD is difficult to assess, the risks and benefits of radiation therapy should be carefully weighed in these patients, particularly if an alternative therapy is available. If there is no alternative, judicious attention to radiotherapy technique may reduce or prevent skin toxicity.
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ranking = 0.125
keywords = vascular disease
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