1/8. A case suggesting lymphocytic vasculitis as a presenting sign of early undifferentiated connective tissue disease.A previously healthy 14-year-old Korean male presented with striking hemorrhagic acral livedo associated with anti-ribonucleoprotein antibodies. His skin biopsy revealed a striking lymphocytic vascular reaction with mild superficial perivascular lymphocytic infiltrates, perivascular extravasation of red blood cells, and leukocytoclastic debris. Lymphocytic vasculitis (LV) is defined in different ways by different authors. The above biopsy findings do not fulfill the criteria for LV of all authors. Chronic lymphocyte-mediated endothelial cell injury may play an important role in the pathogenesis of collagen vascular disease. Cell-mediated cytotoxicity against endothelial cells or other vessel wall components is thought to be the possible pathologic mechanism of LV. We present a patient with early undifferentiated connective tissue disease (EUCTD) with hemorrhagic acral livedo, who finally developed systemic lupus erythematosus. Even though the histopathologic findings do not meet the most rigorous definition of LV, LV may a presenting sign of EUCTD. The purpose of this report is to suggest reconsideration of the diagnostic criteria of lymphocytic vasculitis.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
2/8. Fulminant calcinosis in two patients after kidney transplantation.The first patient had total parathyroidectomy (PTX) with autografting 2 years before. She developed disabling soft-tissue calcifications following kidney transplantation. After reduction of the autograft--a complete removal was not possible--the subcutaneous calcifications regressed. In the second patient, fulminant necrotizing vascular calcinosis developed after successful renal transplantation. Total PTX without autotransplantation was carried out, and progression of the vascular disease was ceased. In both patients, the parathyroid hormone was elevated but not the calcium-phosphate product. We suggest that fulminant calcinosis in patients with kidney transplants requires PTX.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
3/8. prednisolone purpura.Corticosteroids, though known to act effectively against allergy and hypersensitivity, may themselves produce such reactions, ranging from urticaria to anaphylaxis. A middle-aged man with collagen vascular disease developed purpuric rash on repeated administration of steroids. This acute purpuric reaction with sequential colour changes is different than the more common senile purpura-like lesions induced by intake of steroids. It is also different than the purpuric lesions associated with collagen vascular disease per se. The present case favours an allergic aetiology.- - - - - - - - - - ranking = 2keywords = vascular disease (Clic here for more details about this article) |
4/8. Pulmonary and neuromuscular complications of mixed connective tissue disease: a report and review of the literature.We report a 34-year-old woman with mixed connective tissue disease (MCTD) who developed severe pulmonary and neuromuscular complications. At presentation, pulmonary function tests and pulmonary mechanics were suggestive of pulmonary vascular disease, and she subsequently developed clinical signs of pulmonary hypertension. These noninvasive tests may be useful in the timing of more invasive hemodynamic studies. She initially had myasthenia gravis and then developed polymyositis, profound peripheral neuropathy, and ventilatory muscle failure. She died despite aggressive immunosuppressive therapy and plasmapheresis. autopsy showed spinal cord changes secondary to a peripheral neuropathy and signs of neurogenic atrophy confined to the ventilatory muscles. Peripheral neuropathy may be an important cause of ventilatory muscle weakness that can be found in MCTD and systemic lupus.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
5/8. Pulmonary hemosiderosis and immune thrombocytopenia. Initial manifestations of collagen-vascular disease.Severe iron deficiency anemia, pulmonary infiltrates, and cutaneous hemorrhage associated with thrombocytopenia developed in a 7-year-old girl. A typical clinical course and the presence of abundant hemosiderinladen macrophages in the gastric juice and alveoli suggested a diagnosis of idiopathic pulmonary hemosiderosis (PH). Investigation of the marked thrombocytopenia, not previously reported as a finding in IPH, resulted in the demonstration of antiplatelet antibody in the patient's serum and on her platelets. Response to corticosteroid administration and splenectomy was consistent with idiopathic thrombocytopenic purpura (ITP). The IPH and ITP may have occurred coincidentally in this patient, but certain pathophysiological similarities between these two disorders and ultimate development of a poorly defined fatal diffuse connective tissue disorder suggest that thrombocytopenia and intrapulmonary hemorrhage were related.- - - - - - - - - - ranking = 4keywords = vascular disease (Clic here for more details about this article) |
6/8. Colonic manifestations of collagen vascular diseases.Upper gastrointestinal manifestations of collagen vascular diseases have been well described. Recently our attention has been focused on the colonic complications: fibrosis and stricture resulting in obstruction, severe obstipation and recurrent fecal impactions secondary to pseudo-obstruction, progressive colonic dilatation resulting in gangrene of the colon, and sigmoid volvulus and diverticulitis in the presence of both wide- and narrow-mouth pseudodiverticula. patients with these colonic manifestations of collagen vascular disease are disabled, if not severely ill. Recognition of the problem enables the surgeon to plan definitive surgical intervention ranging from segmental resection to total colectomy and ileoproctostomy to restore satisfactory large bowel function.- - - - - - - - - - ranking = 6keywords = vascular disease (Clic here for more details about this article) |
7/8. Psychotic episodes in an elderly woman with an anticentromere-positive scleroderma variant, iga deficiency, and hypothyroidism.A 66-year-old woman presented with a recurrent psychotic disorder with central manifestations that initially suggested a delusional disorder. Peripheral stiffness was at first attributed to a dystonic reaction secondary to neuroleptic treatment. The atypical presentation led to suspicion of collagen vascular disease. Both the psychotic disorder and peripheral involvement responded to cortisone. Findings are discussed in light of recent information on the pathogenesis and neuropsychiatric manifestations of systemic lupus erythematosus, progressive systemic scleroderma and limited scleroderma variants (CREST, unclassifiable connective tissue disease) with positive anticentromere titers, including a discussion of the vasculitis hypothesis of collagen vascular cerebral involvement.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
8/8. Severe back and abdominal pain in a 44-year-old woman.retroperitoneal fibrosis is an uncommon collagen vascular disease. back pain with no specific radiation pattern is a common finding. Evaluation usually begins with an abdominal CT scan or MRI. The finding of fibrous periaortic tissue in conjunction with an elevated erythrocyte sedimentation rate supports the diagnosis. A biopsy is necessary to confirm the diagnosis and exclude malignancy. Sometimes retroperitoneal fibrosis can progress to the point of causing bilateral ureteral obstruction leading to acute renal failure. Corticosteroids, in conjunction with surgery when needed, are the mainstay of therapy.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |