1/74. Infantile disseminated visceral giant cell arteritis presenting as sudden infant death.The rare clinicopathological entity 'disseminated visceral giant cell arteritis' (DVGCA) was first described in 1978. It is characterized by widespread small-vessel giant cell angitis and extravascular granulomas. A normal and healthy 7-month-old boy who presented unexpectedly with sudden infant death syndrome (SIDS) is reported. Histological examination at autopsy revealed giant cell angitis of the aorta, common carotid, coronary, pulmonary, celiac, mesenteric and common iliac arteries. There were also granulomas in the tracheal wall and liver. To our knowledge, this is the first documented case of DVGCA occurring in an infant younger than 12 months of age. A review of the literature on DVGCA is presented in this report, and the differential diagnosis is discussed.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/74. Vasculitis-induced membranous fat necrosis.Membranous fat necrosis (MFN) a distinct degenerative process of adipose tissue, is characterised by the presence of membranocystic lesions (MCLs) superimposed on a background of typical traumatic-type fat necrosis. MCLs are cysts, of varying size and shape, that are lined by an eosinophilic, crenulated membrane, having the staining properties of ceroid. Although MFN has been documented in varying systemic adipose tissue sites and in tumours, the pathogenesis of this pathological curiosity is unknown. To date, an ischemic basis for MFN has been the most proximate, and atherosclerosis and venous insufficiency, due to large and medium vessel disease, have been the most popular underlying clinical disorders. Although systemic vasculitis has been quoted as the underlying ischemic disorder in some patients, vasculitis has not been commented on nor demonstrated in tissue sections in association with MFN. In,reporting vasculitis-induced MFN, we document the occurrence of MFN in association with uncommon causes of vasculitis, namely: 1) Granulomatous vasculitis in a post-herpetic zosteriform scar; 2) cytomegalovirus-induced vasculitis in the clinical setting of systemic lupus erythematosus; and 3) Lymphocytic vasculitis in a tetanus toxoid immunization site reaction.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/74. melkersson-rosenthal syndrome: new clinicopathologic findings in 4 cases.OBJECTIVE: To define the clinicopathologic features of eyelid involvement in melkersson-rosenthal syndrome (MRS). methods: Four patients with eyelid edema consistent with MRS were evaluated clinically, including diagnostic imaging in 2 patients. Eyelid tissue from these patients was examined by light microscopy and immunohistochemistry. polymerase chain reaction for herpes simplex virus was performed in 1 case. RESULTS: The 3 men and 1 woman ranged in age from 33 to 74 years. All patients had insidious, painless, nonpitting eyelid edema. Three patients had unilateral edema; one had bilateral, asymmetric involvement. Ipsilateral lip edema was present in 1 case. Computed tomography demonstrated periorbital heterogeneous thickening that corresponded to the microscopic finding of scattered granulomas. All 4 patients demonstrated epithelioid granulomas inside and adjacent to dilated lymphatic vessels. polymerase chain reaction testing was negative for herpes simplex virus. CONCLUSIONS: Isolated eyelid swelling that mimics thyroid-associated ophthalmopathy may occur in MRS. Computed tomography may be useful in the diagnosis. biopsy should be performed in all cases of unexplained nonpitting eyelid edema. In the eyelid, MRS is characterized histopathologically by a granulomatous lymphangitis, a finding that seems to be unique to this condition.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/74. cerebral amyloid angiopathy (CAA) with presentation as a brain inflammatory pseudo-tumour.cerebral amyloid angiopathy (CAA) is frequent but often asymptomatic. It can induce lobar haemorrhage, rapidly progressive dementia or recurrent transient neurological symptoms, other presentations being less frequent. We report 3 patients in their sixties presenting with a space occupying lesion which was the first manifestation of CAA. They were operated with a diagnosis of cerebral tumour. In all three cases, macroscopy was similar, the lesions were superficial in the cerebral cortex and the preoperative diagnoses were glioblastoma, meningioma and cavernoma. Histologically, the lesions consisted of a large inflammatory granuloma with numerous lipophages and siderophages surrounding capillaries with prominent endothelial cells. Vessels in the near cortex and meninges and within the granuloma harboured heavy amyloid deposits immunolabelled by anti-P component, anti-protein beta A4 with a A40 predominance and anti-apolipoprotein E. Adjacent cerebral cortex showed reactive gliosis and rare senile plaques. amyloidosis is rarely considered among diagnoses of space occupying lesions. In our three cases, CT scan and MRI changes were related to the presence of an inflammatory granuloma around foci of haemorrhage and amyloid laden vessels.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/74. sarcoidosis presenting with large vessel vasculitis and osteosclerosis-related bone and joint pain.A 34-year-old African-American female diagnosed earlier with idiopathic thrombocytopenic purpura (ITP), lymphadenopathy, splenomegaly, uveitis, and pulmonary nodules, developed a subclavian artery aneurysm, and generalized annular osteosclerotic lesions with disabling arthralgias. Biopsies from bone and lymph node revealed non-caseating granulomas and no evidence of malignancy or infection, confirming the clinical impression of sarcoidosis.- - - - - - - - - - ranking = 4keywords = vessel (Clic here for more details about this article) |
6/74. sarcoidosis and systemic vasculitis.BACKGROUND: systemic vasculitis is an unusual complication of sarcoidosis. Over a 10-year period, the authors have provided care for six patients who had features of both sarcoidosis and vasculitis. Vasculitis could not be attributed to other causes. OBJECTIVES: To report six patients (five children) who had sarcoidosis and systemic vasculitis and compare our experience with previous literature. To better delineate the clinical spectrum of sarcoid vasculitis and its response to therapy. methods: Retrospective analysis and a medline literature review of sarcoid and concurrent vasculitis from 1966. RESULTS: Our six patients had systemic illnesses that included fever, peripheral adenopathy, hilar adenopathy, rash, pulmonary parenchymal disease, musculoskeletal symptoms, and scleritis or iridocyclitis. Biopsies revealed features compatible with the diagnosis of sarcoidosis or necrotizing sarcoid granulomata in either skin, lymph node, lung, synovium, bone, bone marrow, liver, trachea, or sclera. Arteriography showed features of large vessel vasculitis in three patients, all of whom were African American, whereas patients with small vessel vasculitis were white. Prior reports of sarcoid and vasculitis included 14 adults, of whom half had predominantly small vessel disease, and half had medium- or large-sized vessel disease. Eight previously reported children included seven with primarily large vessel sarcoid vasculitis. Racial background was noted in 15 reported cases and included whites (6), african americans (5), and Asians (4). Among the authors' six patients, four improved when treated with prednisone alone. However, relapses occurred when the drug was tapered or withdrawn. CONCLUSIONS: sarcoidosis may be complicated by systemic vasculitis that can affect small- to large-caliber vessels. Sarcoid vasculitis can mimic hypersensitivity vasculitis, polyarteritis nodosa, microscopic polyangiitis, or Takayasu's arteritis. African American and Asian patients are disproportionately represented among cases with large vessel involvement. Corticosteroid and cytotoxic therapy is palliative for all forms of sarcoid vasculitis. However, relapses and morbidity from disease and treatment is common.- - - - - - - - - - ranking = 7keywords = vessel (Clic here for more details about this article) |
7/74. Granulomatous osteonecrosis in Crohn's disease.A 25-year-old white woman was diagnosed with Crohn's disease involving the small and large intestines. She had a complex clinical course that required treatment with multiple pharmacological agents, including intravenous, oral and rectal corticosteroids. She also received parenteral nutrition with lipid emulsions. Finally, repeated intestinal resections and drainage of perianal abscesses were required. Her disease was complicated by gallstones, urolithiasis and hip pain. After osteonecrosis was diagnosed, joint replacements were performed. review of the pathological sections from the resected hip, however, resulted in detection of granulomatous inflammation with multinucleated giant cells - the histological 'footprint' of Crohn's disease in the gastrointestinal tract. Because prior specialized perfusion fixation pathological studies of the intestine in Crohn's disease have shown that granulomas are located in the walls of blood vessels, a possible mechanism for the pathogenesis of osteonecrosis in Crohn's disease is chronic microvascular ischemia of bone.- - - - - - - - - - ranking = 7.473979219153keywords = blood vessel, vessel (Clic here for more details about this article) |
8/74. Autoimmune keratolysis in a patient with leukocytoclastic vasculitis: unusual erythema elevatum diutinum with granulomatous pattern.PURPOSE: Leukocytoclastic vasculitis (LCCV) is an immune complex-mediated, small vessel disease that is clinically characterized by the presence of palpable purpuric lesions, most often in association with rheumatic diseases. Ocular manifestations of LCCV are rare. methods: We describe a patient with an unusual granulomatous pattern of erythema elevatum diutinum (EED) associated with autoimmune keratolysis. RESULTS: We studied a 64-year-old man with decreased visual acuity and nodular lesions in both hands. Ocular examination revealed bilateral superior corneal melting with perforation in the left eye and conjunctival thickening in both eyes, in association with a severe inflammatory reaction. Histopathologic examination of the conjunctiva revealed granulomatous vasculitis with neutrophilic infiltrate, giant cells, and fibroblastic proliferation. A punch biopsy taken from his skin showed similar characteristics that suggested EED; however, there were no giant cells. CONCLUSION: To our knowledge, autoimmune keratolysis secondary to cutaneous LCCV (EED) has not been described previously, and there has been no description of granulomatous reaction (in the conjunctiva) in EED.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/74. cholesterol granuloma of the maxillary sinus.cholesterol granuloma is usually associated with chronic middle ear disease and is common in the mastoid antrum and air cells of the temporal bone. It has also been reported in other parts of the skull, such as the frontal and maxillary sinuses and orbit. cholesterol granuloma is rare in the paranasal sinuses. We report a new case of cholesterol granuloma in the maxillary sinus of a 38-year-old man who underwent surgical excision. We also review the literature and discuss the mechanism of development for this lesion. The resected specimen showed fragments of respiratory mucosa with cholesterol clefts surrounded by multinucleated foreign-body giant cells. Some multinucleated foreign-body giant cells showed asteroid bodies. Hemorrhagic areas, hemosiderin-laden macrophages, chronic inflammatory cells, and dilated lymphatics vessels were seen as well. Increased intrasinus pressure due to drainage obstruction may affect venous and lymphatic drainage from the sinus cavity, leading to venule microhemorrhages while still allowing arterial blood into the sinus mucosa and further contributing to a large localized hemorrhage. Lymphatic drainage may be insufficient to completely remove the lipid components of the red blood cells, and the lipid accumulation may contribute to the formation of cholesterol crystals and their esters.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/74. Wegener's granulomatosis: angiographic study and case report with prolonged survival.In the limited form of Wegener's granulomatosis pulmonary arteries were pruned and slightly stretched by a cavitating granuloma. Bronchial and renal vasculature were normal. The microscopic focal small vessel changes are not appreciable at angiography. Therefore, angiography is of no value in establishing the diagnosis of Wegener's granulomatosis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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