1/10. Beware of the heart: the multiple picture of cardiac involvement in myositis.A 42-yr-old woman with dermatomyositis had two myocardial infarctions, episodes of acute chest pain and an acute lung oedema. These events were initially misinterpreted as atherosclerotic ischaemic heart disease accompanying the autoimmune disease. The lack of improvement of cardiac symptoms with anti-ischaemic and immunosuppressive drugs indicated other mechanisms. Intracoronary drug provocation as well as myocardial biopsy revealed a coincidence of small-vessel disease and vasospastic angina as a cause for the severe cardiac symptoms. After initiating therapy with high doses of calcium channel blockers, marked improvement of cardiac symptoms occurred. In the pathogenesis of cardiac involvement in dermatomyositis, two different mechanisms should be considered: inflammatory processes due to dermatomyositis and vasoconstriction caused by an impaired regulation of vascular tone, such as abnormal vessel reactivity or disturbed neuropeptide release. Signs of this generalized vasopathy are Raynaud's phenomenon, Prinzmetal's angina and small-vessel disease, which can coincide. In patients with severe cardiac symptoms and autoimmune diseases, Prinzmetal's angina should be excluded by intracoronary drug provocation using acetylcholine.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/10. A causal role for parvovirus B19 infection in adult dermatomyositis and other autoimmune syndromes.BACKGROUND: infection with parvovirus B19 (B19) has been associated with connective tissue disease (CTD) stigmata, namely, a systemic lupus erythematosus (SLE)-like illness, seronegative polyarthritis resembling rheumatoid arthritis, and vasculitis. The dermatopathology and pathogenetic basis of such B19-associated CTD-like syndromes have not been elucidated. OBJECTIVE: We attempted to document persistence of the B19 genome in skin lesions of 7 patients with CTD-like symptomatology following B19 infection and to correlate systemic manifestations to dermatopathological findings. METHOD: In 7 prospectively encountered patients in whom history, clinical signs and/or serology supported a diagnosis of CTD in the setting of B19 infection, dermatopathological and clinical features were correlated. parvovirus B19 viral genome was sought in skin tissue using the polymerase chain reaction (PCR). RESULTS: Two patients had clinical features diagnostic of myopathic dermatomyositis (DM), 1 of whom is still symptomatic 1.5 years after the onset of her illness, and the other has had typical clinical features of DM for a duration of 3.5 years. A 3rd patient with SLE remains symptomatic 4 years after the onset of her illness. A 4th patient has persistent seronegative symmetrical polyarthritis of 6 years' duration and cutaneous lesions of granuloma annulare (GA). The 5th patient has a 1.5-year history of debilitating polyarthritis and cutaneous lesions with overlap features of DM and subacute cutaneous LE (SCLE). The 6th patient has had a persistent folliculocentric necrotizing vasculitis for 3 years. The 7th patient has a 1-year history of microscopic polyarteritis nodosa (PAN) with cutaneous vasculitis and persistent active renal disease. In 4 patients, exposure to children with fifth disease immediately preceded the onset of their CTD. parvovirus B19 infection was documented serologically in 6 patients with antibodies of IgG subclass in 6 and of IgM subclass in 1. Four of 6 patients questioned had a history of atopy. skin biopsies from patients with clinical features of SLE or DM demonstrated an interface dermatitis with dermal mucinosis. A necrotizing vasculitis with epithelial pustulation was seen in 2 patients. Interstitial GA-like infiltrates were seen in 5 cases. Immunofluorescent (IF) testing revealed a positive lupus band test (LBT) and epidermal nuclear and vascular staining for IgG and C5b-9 in the SLE patient. One DM patient had a negative LBT in concert with C5b-9 deposition along the dermoepidermal junction (DEJ) and within blood vessels while the other showed endomysial vascular Cs5b-9 deposition. In all patients, skin biopsy material contained B19 genome, which was absent in the serum of 4 patients analyzed. Symptomatic relief followed immunosuppressive and immunomodulatory therapy with agents including prednisone, cyclophosphamide, hydroxychloroquine, non-steroidal anti-inflammatory drugs and etanercept, but no patient has had complete symptom resolution. CONCLUSIONS: Persistent B19 infection may be of pathogenetic importance in certain prototypic CTD syndromes, to which underlying immune dysregulation associated with a blunted IgM response to viral antigen may predispose. Anti-viral therapy might be worthy of consideration since traditional immunosuppressive therapy was unsuccessful in our cases.- - - - - - - - - - ranking = 12.004061446077keywords = blood vessel, vessel (Clic here for more details about this article) |
3/10. An autopsy case of intravascular lymphomatosis with dermatomyositis.A 62-year-old man was admitted to hospital with general malaise, arthralgia, edema, and high-grade fever. He was diagnosed as dermatomyositis because of typical skin rashes and muscle weakness. His symptoms were improved by high-doses of prednisolone and cyclosporin A, with the exception of the skin rash over the back. High-grade fever developed again when tapering prednisolone. His condition deteriorated gradually. paresthesia, hypothyroidism, metabolic acidosis, and disseminated intravascular coagulopathy occurred, and he died eight months after the first admission. Postmortem examination revealed intravascular proliferation of atypical mononuclear cells in the lumens of small vessels in all organs. Intravascular lymphomatosis (B cell type) was diagnosed.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
4/10. Primary non-Hodgkin's lymphoma of the transverse colon presenting as dermatomyositis: case presentation and literature review.Primary extranodal non-Hodgkin's lymphoma of the transverse colon is a rare presentation of non-Hodgkin's lymphoma or colonic neoplasm. dermatomyositis is an autoimmune condition of the skin, muscle, and blood vessels that when associated with malignancy is a true paraneoplastic syndrome but is rarely associated with non-Hodgkin's lymphoma. We present a case of primary non-Hodgkin's lymphoma of the transverse colon diagnosed after the presentation of dermatomyositis and review the literature on dermatomyositis and hematologic neoplasm.- - - - - - - - - - ranking = 12.004061446077keywords = blood vessel, vessel (Clic here for more details about this article) |
5/10. Juvenile dermatomyositis: histological findings and pathogenetic hypothesis for the associated skin changes.skin biopsies from patients with scleroderma and juvenile dermatomyositis (DM) share many histologic features. Characteristics common to both diseases are particularly evident in the dermal microvasculature and include endothelial swelling and concentric thickening of the vascular basement membrane. Biopsies performed on 3 patients with the severe vasculitic form of juvenile DM showed these changes as well as dropout of vessels and linear deposition of collagen. The latter findings, seen late in the course of the disease, are indistinguishable from those of advanced scleroderma. A hypothesis is presented which attempts to relate these histological findings to a common underlying pathophysiologic mechanism.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
6/10. dermatomyositis as an immunologic complication of toxoplasmosis.We present immunohistochemical, light- and electron-microscopic findings on a muscle biopsy specimen from a 21-year-old woman who developed debilitating dermatomyosisits in the course of toxoplasmosis. The muscle showed perifascicular muscle cell atrophy and prominent ultrastructural changes consistent with polymyositis. These myopathic changes were interpreted as an immunologic complication of systemic toxoplasmosis and were related to immunohistochemically demonstrable immune complex deposits in the small blood vessels. Our data suggest that dermatomyositis is caused and/or related to toxoplasma infection and is an immune complex-mediated systemic disease.- - - - - - - - - - ranking = 12.004061446077keywords = blood vessel, vessel (Clic here for more details about this article) |
7/10. immunosuppressive agents in corticosteroid-refractory childhood dermatomyositis.Five patients with childhood dermatomyositis, followed for 18 to 96 months, improved when treated with a combination of corticosteroids and methotrexate (one patient) or corticosteroids and cyclophosphamide (four patients) after having become refractory to corticosteroid therapy alone. Complications of vascular involvement in childhood dermatomyositis or from immunosuppressive therapy were observed in most of these patients. Disease components involving skin, muscle, or systemic vessels sometimes varied independently in regard to disease activity or therapeutic responsiveness. In two patients with stable or improving muscle function, cerebrovascular complications occurred. Monitoring of disease activity was best accomplished by clinical evaluation; serum muscle enzymes usually failed to rise prior to or during periods of clinical worsening.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
8/10. Tuberculous infection of skeletal muscle in a case of dermatomyositis.A patient with dermatomyositis associated with carcinoma was treated with steroids and antibiotics for possible tuberculosis. autopsy showed an overwhelming diffuse nongranulomatous infection of mycobacterium tuberculosis involving only the skeletal muscles and one inguinal lymph node. The rare localization of tuberculosis to skeletal muscle in this case is possibly due to steroid immunosuppression and the humoral immune attack on muscle blood vessels that is a part of dermatomyositis.- - - - - - - - - - ranking = 12.004061446077keywords = blood vessel, vessel (Clic here for more details about this article) |
9/10. Small blood vessel involvement in childhood dermatomyositis. An ultrastructural study of a case.The electron microscopic changes in a muscle biopsy from a child with the clinical picture of acute dermatomyositis are described. skin and muscle tissue examined by light microscopy did not revealed vascular alterations. Degeneration of isolated muscle fibers was detected with complete loss of myofilaments and disintegration of the Z-band material. Muscle arteries and veins were free of thrombi, but striking ultrastructural changes were seen in the smallest blood vessels in the muscle. These consisted mainly of vascular occlusion due to necrotic cells mixed with degenerated platelets. Dissoluted endothelial cells and denuded basement membrane were usually seen in the wall of the occluded capillaries. Cytoplasmic tubular particles were present in otherwise unaltered endothelial cells. The possibility that the capillary lesions are related to an acute immunological injury is mentioned.- - - - - - - - - - ranking = 60.020307230383keywords = blood vessel, vessel (Clic here for more details about this article) |
10/10. Tubular cytoplasmic inclusions in a case of childhood dermatomyositis with migratory subcutaneous nodules.A boy with dermatomyositis which began at the age of one year and five months showed multiple migratory subcutaneous nodules, which have seldom been described. Histological and electron microscopic studies of muscles and subcutaneous nodules demonstrated the following interesting findings. 1. light microscopy. The migratory subcutaneous nodules consisted of non-suppurative panniculitis and ischaemic adipo-necrosis as a sequel to vascular lesions. This finding suggests that the nodules may have arisen from the subcutaneous adipose tissue which had been severely damaged by systemic angiopathy. 2. Electron microscopy. Examination of the vessels in muscle and subcutaneous nodules showed tubular cytoplasmic inclusions with a diameter of approximately 250 A in the endoplasmic reticulum of vascular endothelial cells. These observations provide strong support for the concept that the fundamental pathologic process in childhood dermatomyositis is of a vascular nature, and the primary lesion is in the walls of the intramuscular blood vessels.- - - - - - - - - - ranking = 12.33739477941keywords = blood vessel, vessel (Clic here for more details about this article) |
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