1/252. Cutaneous polyarteritis nodosa: a case report and literature review.Cutaneous polyarteritis nodosa (CPN) is an uncommon form of vasculitis. It exists as a separate entity, though bearing similar name with polyarteritis nodosa (PAN) which is an aggressive systemic vasculitis with multi-organ involvement. CPN runs a chronic but benign course. Its aetiology is unknown and it usually presents with painful nodules on the legs with mild constitutional symptoms, and extracutaneous features of arthralgias, arthritis, neuropathy and myopathy. No mortality has been reported thus far. It is therefore important to distinguish CPN apart from PAN. Symptomatic treatment with judicial use of systemic steroids and anti-inflammatory agents will suffice in most cases.- - - - - - - - - - ranking = 1keywords = vasculitis (Clic here for more details about this article) |
2/252. Familial occurrence of systemic vasculitis and rapidly progressive glomerulonephritis.Two familial clusters of systemic vasculitis are described. In one family, microscopic polyangiitis and rapidly progressive glomerulonephritis occurred in HLA-identical siblings; in the second family, 3 second- and fourth-degree related members were affected by Wegener's granulomatosis. Published clusters of systemic vasculitides and Goodpasture's syndrome are reviewed, and, together with the observed families, the evidence for genetic susceptibility and a causative role of environmental factors for these diseases with special emphasis on the HLA system is discussed.- - - - - - - - - - ranking = 2.5keywords = vasculitis (Clic here for more details about this article) |
3/252. Necroqizing enterocolitis with pneumatosis intestinalis in systemic lupus erythematosus and polyarteritis.Pneumatosis intestinalis was encountered in association with fatal necrotizing enterocolitis in systemic lupus erythematosus (SLE) and polyarteritis nodosa. The radiologic identification of mottled, bubbly, and linear collections of intramural intestinal gas distinguish this ominous complication from benign pneumatosis cystoides intestinalis. In the setting of intestinal vasculitis due to SLE or polyarteritis nodosa, these characteristic radiologic features indicate necrotizing enterocolitis. Since corticosteroids may mask clinical progression of the intestinal lesion, radiologic evaluation is essential in the overall management of the patient with intestinal vasculitis.- - - - - - - - - - ranking = 1keywords = vasculitis (Clic here for more details about this article) |
4/252. Periarteritis nodosa presenting as a breast lesion: report of a case and review of the literature.We describe a 34-year-old woman with periarteritis nodosa (PAN) presenting as a breast lesion. Localized involvement of the breast is an unusual manifestation of PAN. To date, 10 cases have been reported: all were in women with an age range of 45-78 years (mean 63). In most cases, breast lesions were an isolated finding, and the prognosis was favourable, setting them apart from the more common form of systemic PAN. The case presented is unusual in that vasculitis developed in the postpartum period, and was associated with cutaneous PAN-like lesions elsewhere on the body, and digital artery occlusion. The most important differential diagnoses of PAN of the breast are infectious mastitis, mammary malignancy and other forms of idiopathic vasculitides of the breast, e.g. giant cell arteritis and wegener granulomatosis.- - - - - - - - - - ranking = 0.5keywords = vasculitis (Clic here for more details about this article) |
5/252. Early lacunar strokes complicating polyarteritis nodosa: thrombotic microangiopathy.OBJECTIVE: To determine the patterns and mechanisms of polyarteritis nodosa (PAN)-associated strokes (PANAS). BACKGROUND: Strokes are reputed to be rare complications of PAN and to occur at a late stage (2 to 3 years). The cause of stroke is unknown but may be related either to atherosclerosis-like occlusive vasculopathy, caused possibly by hypertension or corticosteroid (CS) use, or to vasculitic arterial occlusion. methods: Clinical and radiologic patterns, latencies, and current therapy at onset in 15 PANAS patients (4 of the authors' and 11 published cases) were analyzed. RESULTS: A lacunar stroke syndrome (11/15 cases, 73%) was the most frequent stroke pattern in PANAS (multiple, small, deep infarcts in 6, [55%], pontine lacunae in 3 [27%], and leukoaraiosis in 2 [18%]), followed by pure lobar hematoma and bilateral, possibly cardioembolic, large ischemic infarcts (2 cases each). A stroke latency shorter than that previously established (within 8 months in 73% of cases; mean latency, 6.5 months) and a close relationship between the use of CS and stroke in PAN also were found. Of the 77% of first-time or recurrent lacunar strokes that developed despite CS therapy, 80% appeared within 6 months and 50% within 3 weeks of CS initiation. CONCLUSION: Early lacunar stroke syndrome, related to deep small- or pontine-penetrating artery thrombotic microangiopathy rather than vasculitis, was the most frequent PANAS pattern. This vasculopathy may be aggravated by corticosteroid (CS) therapy enhancement of either platelet thromboxane a2 production or arterial wall fibrosis. Thus, antiplatelet drugs in association with CS may be advisable for preventing stroke occurrence or recurrence in PAN.- - - - - - - - - - ranking = 0.5keywords = vasculitis (Clic here for more details about this article) |
6/252. Acquired bernard-soulier syndrome: a case with necrotizing vasculitis and thrombosis.We describe a patient with positive antinuclear antibodies, polyclonal gammopathy and high level of circulating immunocomplexes, resulting in vascular purpura. In addition, the patient had a slightly prolonged bleeding time and an isolated defect of ristocetin-induced platelet aggregation (RIPA) in platelet-rich plasma (PRP). The patient's plasma also inhibited RIPA in normal PRP and in normal platelet suspension. The activity and multimeric structure of plasmatic von willebrand factor showed no alteration. We could demonstrate an autoantibody against platelet membrane glycoprotein (GP) Ib, using an ELISA-type assay. These data suggest an acquired bernard-soulier syndrome. We suggest that the patient had an immunocomplex-mediated leukocytoclastic vasculitis accompanied by production of antinuclear autoantibodies as well as the presence of an autoantibody against GPIb. The titer of the anti-GPIb antibody, however, was too low to induce significant platelet-type bleeding tendency, only laboratory alterations were found. Moreover, in a later stage of her disease, she developed a severe necrotizing vasculitis which was followed by a deep venous thrombosis.- - - - - - - - - - ranking = 3keywords = vasculitis (Clic here for more details about this article) |
7/252. Immune-mediated pathology following hepatitis B vaccination. Two cases of polyarteritis nodosa and one case of pityriasis rosea-like drug eruption.The association of hepatitis b virus infection and vasculitis or other immune-mediated manifestations is well documented. Reports on such manifestations in relation to hepatitis B vaccination are scarce, however. We report 2 patients who developed polyarteritis nodosa following vaccination against hepatitis B. In one patient this resulted in an ischemic and necrotic digital ulcus, necessitating surgical amputation. The other patient presented with typical cutaneous polyarteritis nodosa which responded well to corticosteroid treatment. A third patient developed a severe pityrias rosea-like eruption. He was treated with topical steroids with healing of the lesions, leaving only post-inflammatory hyperpigmentation. The literature on these associations is reviewed.- - - - - - - - - - ranking = 0.5keywords = vasculitis (Clic here for more details about this article) |
8/252. Endoneurial vasculitis and tubuloreticular inclusions in peripheral nerve biopsy.We describe 3 patients in whom nerve biopsy revealed endothelial tubuloreticular inclusions in association with peripheral nerve endoneurial vasculitis. Two of the patients had systemic lupus erythematosus (SLE), while the third was hiv-positive. review of our biopsy material featuring the much more common finding of epineurial vasculitis failed to disclose any instances in which endothelial tubuloreticular inclusions (TRIs) were present. We conclude that TRIs and endoneurial vasculitis are closely associated. Moreover, if detected on a nerve biopsy specimen, TRIs are very suggestive of SLE or hiv infection. Finally, literature evidence is cited to suggest that an "acid-labile" alpha-interferon may be pathogenically related to the vasculitic process in these patients, perhaps through a process mediated by tumor necrosis factor.- - - - - - - - - - ranking = 3.5keywords = vasculitis (Clic here for more details about this article) |
9/252. polyarteritis nodosa presenting as massive upper gastrointestinal hemorrhage.We report the first case of massive upper gastrointestinal hemorrhage as the initial presentation of polyarteritis nodosa (PAN), which is an uncommon form of systemic necrotizing vasculitis that may involve many organ systems and could affect any age group. abdominal pain is the most common sign of gastrointestinal involvement. Gastrointestinal bleeding occurs less frequently in approximately 6% of cases. Reported cases of gastrointestinal hemorrhage have been in the form of coffee ground emesis, melena, or hematochezia. Such bleeding complications have resulted from ischemic ulceration or perforation of the small or large intestine. However, we are unaware of previous reports showing massive hematemesis to be the initial presentation of PAN.- - - - - - - - - - ranking = 0.5keywords = vasculitis (Clic here for more details about this article) |
10/252. (A)symptomatic necrotizing arteritis of the female genital tract.AIMS: The vasculitides represent a heterogenous set of disorders that differ in prognosis and response to therapy. Beside systemic vasculitides, the development of localized forms of arteritis is well known though uncommon and the etiopathogenesis is not yet definitely clear. methods: patients with necrotizing arteritis of the female genital tract proven by histology are studied in a retrospective analysis. RESULTS: Three cases of necrotizing arteritis with histological features of panarteritis nodosa apparently confined to the female genital tract are presented. None of these patients had prior history of systemic vasculitis. The acute necrotizing vasculitis was confined only to the uterine cervix in two patients and involved all the internal genital organs in the third patient. The patients have been observed for up to 4 years without any therapy for these lesions and without any manifestation of systemic vasculitic progression. CONCLUSION: It is to speculate that focal arteritis of the female genital tract is a benign form of panarteritis nodosa or moreover a totally different entity with identical morphogenesis but possibly different pathogenesis. Furthermore it seems to be important to be aware of the specificity of focal arteritis in female genital tract as distinct from the generalized form to prevent unnecessary surgical or chemotherapeutical therapy for this lesion. The benign entity of local arteritis in the female genital tract is discussed in contrast to the severe prognosis of systemic panarteritis nodosa.- - - - - - - - - - ranking = 1keywords = vasculitis (Clic here for more details about this article) |
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