Cases reported "Collagen Diseases"

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1/167. Pneumatosis intestinalis in association with connective tissue disease.

    Pneumatosis intestinalis in association with connective tissue diseases is an unusual combination whose pathogenesis is not yet understood. Furthermore, steroid medication, often used to treat these diseases, may itself cause pneumatosis. Three cases of scleroderma, systemic lupus erythematosus, and amyloidosis in association with pneumatosis and without prior steroid therapy are presented. The small vessel occlusive pathologic processes in these diseases may cause focal areas of mucosal ischemia resulting in small, perhaps transient ulcerations that allow gas to enter the gut wall from the lumen. ( info)

2/167. Can immunoadsorption plasmapheresis be used as the first choice therapy for neuroimmunological disorders?

    The subjects were 31 patients in whom immunoadsorption plasmapheresis (IAPP) was performed as the first choice therapy for primary or recurrent neuroimmunological disorders. The clinical manifestations before and after IAPP and the use of corticosteroids were investigated in the present study. IAPP was clinically effective in all patients. The corticosteroids (CSs) administration was begun or CSs were increased after completion of IAPP in 11 patients. IAPP was performed as the first choice therapy, and favorable results were obtained in patients with guillain-barre syndrome and Miller-Fisher syndrome. IAPP alone was also effective in a patient with lupoid sclerosis. When IAPP was used alone in 2 patients with chronic inflammatory demyelinating polyradiculoneuropathy, it completely eliminated the clinical manifestations, but the symptoms recurred about 2 months later. Therefore, although IAPP could be performed as the first choice therapy for many neuroimmunological disorders, subsequent therapies should be carefully investigated. ( info)

3/167. Anitschkow cells in the human heart.

    A case of multiple myeloma, who died of congestive heart failure of unknown cause, is presented. Microscopically, Anitschkow cells with caterpillar and owl-eyed nuclei were scattered in the interstitium of the myocardium. To know the pathological significance of their appearance, histological observations of normal, developing and autoimmune disease-bearing heart specimens were made. A few Anitschkow cells were commonly seen in the interstitium of the adult heart with or without autoimmunity, and fetal cardiomyocytes often showed Anitschkow-type nuclear configurations. Moreover, squamous cancer cells metastatic to the myocardium exhibited Anitschkow-type nuclei. No Anitschkow-type nuclei were observed in extracardiac locations. In conclusion, formation of Anitschkow-type nuclear features in the human heart is not necessarily specific to the autoimmune status, but can be regarded as being ubiquitously provoked by the continuous contraction of heart muscle cells. ( info)

4/167. Acquired reactive perforating collagenosis associated with diabetes mellitus: eight cases that meet Faver's criteria.

    Reactive perforating collagenosis (RPC) is characterized by umbilicated papules with a central adherent keratotic plug. Histologically, this condition shows transepidermal elimination of altered dermal collagen bundles into a cup-shaped epidermal depression. The present paper describes eight patients with associated diabetes mellitus who meet the diagnostic criteria for the acquired form of RPC (ARPC). Although half of these patients underwent dialysis, the lesions did not tend to develop after dialysis. pruritus and the Koebner phenomenon were common, and histologically a microvasculopathy was noted in the dermis of all patients. We speculate that this disease is triggered by a cutaneous response to superficial trauma. Furthermore, this response acts synergistically with vasculopathy in the dermis, primarily in the case of diabetes mellitus. A secondary sign of ARPC may be degenerated collagen fibres as a result of transepidermal elimination. ( info)

5/167. Collagenous gastritis and collagenous colitis: a report with sequential histological and ultrastructural findings.

    The case is reported of a young adult man with collagenous gastritis, an extremely rare disorder with only three case reports in the English literature, who subsequently presented with collagenous colitis. Sequential gastric biopsies showed a notable increase in thickness of the subepithelial collagen band. Ultrastructural study of gastric and rectal mucosa showed the characteristic subepithelial band composed of haphazardly arranged collagen fibres, prominent degranulating eosinophils, and activated pericryptal fibroblasts. ( info)

6/167. collagen vascular diseases and radiation therapy: a critical review.

    PURPOSE: Although many oncologists have the impression that patients with collagen vascular disease tolerate radiotherapy less well than other patients, until now this was never described in a review article. methods AND RESULTS: The principal objective was to determine whether patients with collagen vascular diseases have a greater risk of severe radiation therapy complications, than those without a collagen vascular disease. However, most of the publications found on this topic are short anecdotal case reports of patients with increased toxicity after radiation. Consequently, the true incidence of these side effects is unknown. CONCLUSIONS: Unless further studies on this subject are reported, each radiation oncologist should be cautious in treating these patients. ( info)

7/167. Late onset focal dermal elastosis: a distinct clinicopathologic entity?

    Two patients, aged 72 and 69 years, had skin lesions clinically resembling pseudoxanthoma elasticum (PXE). On histologic examination, the lesions showed an increased accumulation of normal-appearing elastic fibers lacking the abnormalities that characterize PXE. Similar lesions have been described previously in two elderly patients by Tojima et al. (1995). The clinical and histologic differential diagnosis of these lesions is discussed, and it is concluded that they represent a clinicopathologic entity with undetermined prevalence and etiopathogenesis. ( info)

8/167. Fibrogenesis imperfecta ossium: imaging correlation in three new patients.

    OBJECTIVE: Fibrogenesis imperfecta ossium is an extremely rare disorder that can easily be misdiagnosed. We retrospectively reviewed the clinical and imaging data of three confirmed cases of fibrogenesis imperfecta. DESIGN AND patients: The patients consisted of two men and one woman, ranging in age from 40 to 53 years. radiography was performed in all the patients. One patient had a 3-year follow-up of the thoracolumbar spine with conventional radiography and thoracolumbar magnetic resonance (MR) imaging. Open biopsy was performed in all cases, confirming the diagnosis of fibrogenesis imperfecta ossium. RESULTS: All our cases demonstrated "fishnet" trabecular pattern by conventional radiographs, and a pelvis radiograph of one patient showed an equivocal sclerosis pattern. Multiple fractures were noted in two patients. A pseudoexostosis was present in the ilium in one patient. Thoracolumbar MR imaging demonstrated diffuse low signal intensity within the medullary space on both T1-weighed and T2-weighted images, except for a region of increased signal intensity in the L1 and L2 vertebral bodies on T2-weighed images due to edema from acute collapse. CONCLUSIONS: Although uncommon, fibrogenesis imperfecta ossium should be considered in a previously healthy patient with a combination of progressive bone pain, unexplained fractures, a radiologic pattern of fishnet osteopenia and MR imaging of low signal intensity bone marrow on both T1-weighted and T2-weighted images. ( info)

9/167. Severe hypopharyngeal dysphagia in a patient on chronic steroid treatment.

    A 44-year-old Caucasian male who had been on long-term steroid treatment for an unspecified collagen disease was referred to our Unit because of fever, severe hypopharyngeal dysphagia, night sweats and evidence of marked superior vena cava compression. Extrapulmonary disseminated tuberculosis also involving bone and liver was eventually diagnosed and proven by means of specific polymerase chain reaction assay. Antimycobacterial treatment, which led to a dramatic improvement within two months, was protracted for 18 months until the complete return to normal of both clinical and laboratory findings. ( info)

10/167. Treatment of pruritis of reactive perforating collagenosis using transcutaneous electrical nerve stimulation.

    Reactive perforating collagenosis is a form of perforating dermatosis due to transepithelial elimination of collagen and characterized by itchy papulonodular eruptions frequently seen in patients with diabetes mellitus and end stage renal failure. pruritus is often severe and treatment is difficult. Two adult Chinese diabetic patients with acquired reactive perforating collagenosis unresponsive to topical therapies and oral antihistamines, were treated with transcutaneous electrical nerve stimulation. There was a significant reduction of itch followed by gradual resolution of the skin lesions. ( info)
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