1/19. Cogan's syndrome: organ-specific autoimmune disease or systemic vasculitis? A report of two cases and review of the literature.Cogan's syndrome is a rare disorder of unknown origin characterized by inflammatory eye disease and vestibuloauditory symptoms. Typically, young adults suffer from interstitial keratitis and sudden onset of tinnitus and hearing loss. Few cases (around 150) have been published and thus it is difficult to determine the percentage of patients with underlying systemic disorders such as systemic vasculitis. The variety of systemic manifestations is large and includes fever, splenomegaly, lymphadenopath, and musculoskeletal complaints. systemic vasculitis can be seen in around 10% of cases and may involve the large vessels, appearing as Takayasu-like vasculitis with affection of the aortic valve but also the coronary arteries and the small kidney vasculature. Evaluating the exact extension of the systemic features determines the choice of treatment. While corticosteroids have proved to be of short-term benefit, long-term treatment with immunosuppressive drugs is controversial. Auditory function in deaf patients has often been restored successfully with cochlear implants. To illustrate the nature of the syndrome, we present two patients with a wide clinical spectrum of symptoms from local disease restricted to the eyes and ears to a widespread vasculitis affecting arteries of the brain, kidney and the upper and lower extremities. We then review the typical aspects as well as the etiology of the disease.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/19. hepatitis c, cryoglobulinemia, and cutaneous vasculitis associated with unusual and serious manifestations.hepatitis c viral infection is currently the leading cause of chronic hepatitis and cirrhosis. It also is a major predisposing factor for the development of hepatocellular carcinoma. It is estimated that approximately 1-2% of patients with hepatitis c infection have nonhepatic manifestations that are protean in nature. In this report, we describe six unusual cases of nonhepatic manifestations: abdominal vasculitis in two, peripheral neuropathy in two, and one patient each with central nervous system vasculitis and necrotizing cutaneous vasculitis. All patients had cutaneous vasculitis and cryoglobulinemia. None of our patients had cirrhosis, yet three of the six patients died. Because of the severe manifestations, aggressive therapy was instituted with interferon, immunosuppressive medications, i.v. immunoglobulin, and plasmapheresis. Our report underscores the importance of recognizing nonhepatic manifestations in patients with hepatitis c infection that may be associated with high morbidity and mortality.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/19. Sudden death due to unsuspected coronary vasculitis.Coronary artery vasculitis is a well-recognized complication of polyarteritis nodosa and is occasionally seen in other forms of systemic vasculitis. However, involvement of the major epicardial coronary arteries leading to myocardial infarction and death is uncommon. Isolated coronary arteritis is even more rare. We report three cases of sudden death due to myocardial ischemia associated with arteritis of the major coronary arteries. All three decedents were previously healthy young to middle-aged men who had died suddenly after complaints of chest pain and shortness of breath. The autopsy findings and differential diagnoses are presented. Such cases are of particular interest to the medical examiner because of the sudden, unexpected nature of the deaths. An approach to the correct diagnosis is discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/19. Granulomatous angiitis of the spinal cord associated with Hodgkin's disease.A 28-year-old man had a 5-month history of focal and generalized neurologic symptoms culminating in a thoracic myelopathy. Evaluation revealed granulomatous angiitis of the spinal cord in association with occult nodular sclerosing Hodgkin's disease. In previous reports, manifestations indicative of intracranial involvement have dominated the clinical presentation of granulomatous angiitis associated with Hodgkin's disease. Successful therapy for Hodgkin's disease may result in marked improvement of associated granulomatous angiitis, whereas the lack or failure of therapy results in a uniformly fatal outcome. Definitive antemortem diagnosis of granulomatous angiitis requires a biopsy of involved tissue. The cause of granulomatous angiitis, as well as the nature of its association with Hodgkin's disease, remains unexplained.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/19. Visual loss with dancing extremities and mental disturbances.A case is reported of a teenage girl, who presented with a profound loss of vision in the right eye, secondary to retinal vasculitis. During the preceding year, a gradual change in her personality had been noted, associated with a deterioration in her intellect. She developed an area of pigment epithelial disturbance in the macular region of the right eye, and subsequently, optic atrophy. One year after this, she had a similar, but more localized episode in the left eye. She later developed involuntary movements, and two years after initial presentation, a diagnosis of subacute sclerosing panencephalitis was made. Subsequently her clinical condition deteriorated rapidly and she died. Post-mortem examination confirmed the diagnosis of subacute sclerosing panencephalitis. This case demonstrates the insidious nature of the disease and suggests that the diagnosis of subacute sclerosing panencephalitis should be considered in any child or young adult who presents with unexplained retinal vasculitis, maculopathy or chorioretinitis.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/19. Angiotropic lymphoma with histologic features of neoplastic angioendotheliomatosis presenting with predominant respiratory and hematologic manifestations. Report of a case and review of the literature [corrected]Neoplastic angioendotheliomatosis (NAE) is a rare fatal disease characterized by widespread intravascular proliferations of neoplastic mononuclear cells. Clinically, dermatologic and bizarre neurologic manifestations usually predominate. The origin of the neoplastic cells remains still undetermined. The authors report a patient with NAE peculiar with respect to the following points: (1) the patient predominantly manifested respiratory symptoms and hematologic findings and lacked cutaneous or neurologic manifestations; and (2) immunohistochemical and molecular genetic studies showed the B-cell nature of the neoplastic cells, although previous cases with predominant respiratory or hematologic manifestations were reported to be of endothelial origin. Despite the rarity, this type of NAE or angiotropic [corrected] lymphoma should be recognized because it is easily confused with other disorders, particularly vasculitis or thrombotic thrombocytopenic purpura.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/19. dapsone is an effective therapy for the skin lesions of subacute cutaneous lupus erythematosus and urticarial vasculitis in a patient with C2 deficiency.The deficiency of second component of complement (C2d) is the most common hereditary complement deficiency. patients with C2 deficiency are frequently associated with an auto-immune disease process, in particular, systemic lupus erythematosus (LE)-like syndrome and/or vasculitic syndrome or bacterial infections. C2d has been associated with the LE subset of subacute cutaneous LE (SCLE), the presence of anti-Ro (SSA) antibodies, and the human leukocyte antigen (HLA) types A10, B18, DR2. We describe the clinical, serologic and immunogenetic data in a patient with manifestations of sjogren's syndrome who developed urticarial vasculitis and photosensitive annular SCLE which were effectively treated with oral dapsone. Our case illustrates the dynamic nature of LE.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/19. Radionuclide scanning in pseudo-Kaposi's sarcoma.sodium pertechnetate tc 99m radionuclide scanning is considered to be a sensitive indicator in detecting lesions of Kaposi's sarcoma, including occult infiltrations. This report describes a "false-positive" scan in a patient with acroanglodermatitis (pseudo-Kaposi's sarcoma); therefore, this technique may not be useful in the differential diagnosis of these two clinically and histologically similar diseases. We suggest, instead that the technetium scan may be helpful in deciding on the course of therapy in acroangiodermatitis by demonstrating the nature of the underlying vascular anomalies.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/19. Ankylosing spondylitis with subsequent development of rheumatoid arthritis, sjogren's syndrome, and rheumatoid vasculitis.Twelve years after the onset of ankylosing spondylitis, a patient developed severe seropositive rheumatoid arthritis with subcutaneous nodules. This was subsequently complicated by sjogren's syndrome and rheumatoid vasculitis. The literature is reviewed, and the nature of the association between ankylosing spondylitis and rheumatoid arthritis is discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/19. Pulmonary disease in idiopathic urticarial vasculitis.A 45-year-old woman developed chronic urticaria clinically and cutaneous necrotizing vasculitis histologically, with arthralgias, abdominal pain, angioedema, hemoptysis, and pleuritic chest pain. An open lung biopsy revealed a leukocytoclastic vasculitis of the pulmonary venules, which may be etiologically associated with the chronic obstructive lung disease detected in this patient by pulmonary function tests. The documentation of pulmonary disease in patients with urticarial vasculitis emphasizes the potentially serious nature of this disorder and the need for careful diagnosis and prompt, vigorous treatment.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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