1/144. Aneurysms and hypermobility in a 45-year-old woman.EDS type IV presents a diagnostic and therapeutic challenge to the primary care physician, surgeon, and rheumatologist. In patients for whom the diagnosis is known, avoidance of trauma, contact sports, or strenuous activities, joint bracing and protection, and counseling on contraception are helpful preventive strategies. In patients presenting with vascular, gastrointestinal, or obstetric complications, a history of hypermobility and skin fragility (easy bruising, abnormal scarring, poor wound healing) should lead to a suspicion of this diagnosis, and to caution in the use of certain invasive diagnostic and operative techniques. Efforts should be made to examine family members. Most importantly, when caring for such patients, the acute onset of headaches, chest pain, shortness of breath, and abdominal pain should arouse suspicion of a potentially catastrophic vascular or visceral event.- - - - - - - - - - ranking = 1keywords = abdominal pain, headache, chest (Clic here for more details about this article) |
2/144. purpura as a cutaneous association of sickle cell disease.A common chronic feature of sickle cell disease is the presence of painful, punched-out leg ulcers. Other cutaneous findings in patients with homozygous sickle cell disease have not been described in the literature. We present a case of a 50-year-old black woman with sickle cell disease who was admitted for acute onset of arm and hip pain. After admission she deteriorated clinically, with multiorgan failure and mental status changes. Examination of the skin revealed erythematous papules and plaques with scaly centers and purpura on the upper trunk. The clinical differential diagnosis was vasculitis versus sepsis. skin biopsy of two representative lesions was performed. hematoxylin- and eosin-stained sections showed a superficial perivascular mixed inflammatory infiltrate with numerous eosinophils and extravasated erythrocytes, some of which exhibited bizarre morphology of sickled red blood cells. These findings indicated that the patient's cutaneous lesions, possibly multifactorial in origin, could be a component of her sickle cell crisis. This case is presented as an unusual one in which evaluation of erythrocyte morphology contributed to patient management and to emphasize the importance of examining erythrocyte morphology as a part of the histologic evaluation of stained tissue.- - - - - - - - - - ranking = 0.019026616404241keywords = upper (Clic here for more details about this article) |
3/144. Diaphragmatic paresis as a manifestation of large artery vasculitis.We describe the initial presentation and followup of a 54-year-old Caucasian woman who presented in 1995 with bilateral arteritis of the axillary arteries and acute onset dyspnea. Chest radiograph, chest fluoroscopy, and pulmonary function studies confirmed the diagnosis of right hemidiaphragmatic paresis. Prednisolone and methotrexate therapy and short term anticoagulation were initiated and she experienced no further sequelae during 2 years of followup.- - - - - - - - - - ranking = 0.023615938094468keywords = chest (Clic here for more details about this article) |
4/144. Pustular vasculitis of the hands.A case of pustular vasculitis of the hands with evidence of systemic involvement is described. A 64-year-old woman presented with a 2-day history of large, tense bullae arranged symmetrically over the dorsum of the three radial digits and extending on to the radial aspect of the dorsum of each hand. The bullae caused some discomfort and prevented normal use of her hands. There was no response to antibiotic therapy initiated prior to referral to hospital. Initial investigations revealed a raised white cell count with a neutrophilia, a raised erythrocyte sedimentation rate and a raised c-reactive protein. Abnormalities of liver function were detected. Aspirates from the bullae and blood cultures were sterile. The histology of debrided tissue demonstrated a florid neutrophilic dermal infiltrate with many blood vessels associated with prominent fibrin. A diagnosis of pustular vasculitis of the hands was made. The bullae were surgically debrided and treatment with oral corticosteroids was started. Two days after commencement of oral prednisolone, a crusted pustule appeared on her upper cutaneous lip. There was rapid resolution of both the vasculitis of the hands and the pustule on her upper lip following an increase in the dose of oral prednisolone. The patient was discharged on the seventeenth day following admission.- - - - - - - - - - ranking = 0.038053232808483keywords = upper (Clic here for more details about this article) |
5/144. De novo ANCA-associated vasculitis occurring 14 years after kidney transplantation.A cadaveric kidney transplant recipient, with no history of a connective tissue disease, was admitted with malaise, arthralgias, diplopia, mild headache, and a painful left eye. The patient was on maintenance immunosuppression for 14 years with cyclosporine and methylprednisolone. Initial laboratory data indicated an elevated serum creatinine from baseline, 2 proteinuria, and 50 to 100 red blood cells (RBCs)/high-power field (HPF) in the urine. Renal biopsy was consistent with necrotizing vasculitis involving glomerular capillaries, with crescent formation and an absence of immune complexes. Perinuclear antineutrophil cytoplasmic autoantibodies (P-ANCA) and anti-myeloperoxidase (MPO) were found to be elevated. To the best of our knowledge, this is the first reported case of an ANCA-associated small vessel vasculitis (SVV) developing in a renal transplant recipient without history of connective tissue disease.- - - - - - - - - - ranking = 0.32429555543009keywords = headache (Clic here for more details about this article) |
6/144. Rheumatoid arthritis accompanied by colonic lesions.A 69-year-old woman with a 6-year history of rheumatoid arthritis treated solely with an orally administered NSAID had slowly progressing persistent mild abdominal pain and diarrhea, accompanied with marked sing of inflammation as well as hypoproteinemia due to protein-losing gastroenteropathy. Examinations of the large intestine revealed variously shaped ulcerative lesions, centered around the left hemicolon, as well as luminal narrowing. The course of the disease and the shape of the lesions strongly suggested involvement of rheumatoid vasculitis; oral administration of prednisolone was effective.- - - - - - - - - - ranking = 0.65208850647544keywords = abdominal pain (Clic here for more details about this article) |
7/144. systemic vasculitis with bilateral perirenal haemorrhage in chronic myelomonocytic leukaemia.The cases of two patients with chronic myelomonocytic leukaemia associated with periarteritis nodosa-like, antineutrophil cytoplasmic antibody negative, systemic vasculitis, are reported. A 61 year old man was admitted with fever, diffuse myalgia, and abdominal pain. Blood and bone marrow examination showed chronic myelomonocytic leukaemia. Vasculitis of the gall bladder was responsible for acalculous cholecystitis. A massive spontaneous bilateral perirenal haemorrhage occurred. A 73 year old woman with chronic myelomonocytic leukaemia had been followed up for one year when unexplained fever occurred. Two months after the onset of fever, sudden abdominal pain was ascribed to spontaneous bilateral renal haematoma related to bilateral renal arterial aneurysms. Neuromuscular biopsy showed non-necrotising periarteriolar inflammation. To our knowledge, systemic vasculitis has never been reported in chronic myelomonocytic leukaemia. In our two cases a non-random association is suggested because (a) chronic myelomonocytic leukaemia is a rare myelodysplastic syndrome, (b) spontaneous bilateral perirenal haematoma is not a usual feature of periarteritis nodosa.- - - - - - - - - - ranking = 1.3041770129509keywords = abdominal pain (Clic here for more details about this article) |
8/144. Giant cell angiitis of the central nervous system with amyloid angiopathy. A case report and review of the literature.We report a new case of giant cell angiitis of the central nervous system associated with cerebral amyloid angiopathy (GA/CAA). A 67-year-old woman was hospitalized with a history of headaches and lapses of consciousness. After improvement with corticosteroidtherpay, treatment was stopped. She relapsed and died 33 days after first admission. Pathological examination showed unusual extension of GA/CAA lesions, in the superficial and deep layer of the cerebral cortex, and in the cerebellum. Simultaneous occurrence of GA and CAA is rare. Histopathologic findings and immunological pathogenesis of the process are discussed: 1) arguments over pre-existence of CAA, responsible for GA; 2) primitive inflammatory process inducing amyloid deposits; 3) GA/CAA may represent an association of histological lesions related to 2 different types of disease: i) neurodegenerative disease with specific lesions (such as presence of diffuse senile plaques and neurofibrillary tangles) inducing inflammatory reaction ii) inflammatory disease, with few or no degenerative lesions, responding to immunotherapy.- - - - - - - - - - ranking = 0.32429555543009keywords = headache (Clic here for more details about this article) |
9/144. Acute meningococcaemia complicated by late onset gastrointestinal vasculitis.Extra-meningeal and non-cutaneous manifestations of meningococcal infection are well recognized but rare. Herein we report a little recognized complication of meningococcaemia, namely late-onset gastrointestinal vasculitis. In the case presented, the prostacycline analogue iloprost was used in an attempt to minimize incipient digital gangrene ina patient with evidence of protracted immunological phenomena. In addition, diclofenac was used to treat severe arthralgia. Withdrawal of iloprost on day 19 was associated with abdominal pain and peritonism. laparotomy demonstrated ischaemia and ulceration in the caecum, and histology revealed full thickness mucosal ulceration with a prominent vasculitic process. It is postulated that these findings were immunologically mediated, with possible rebound vasoconstriction following withdrawal of iloprost. It is also possible that NSAID use contributed to the findings via cyto-toxicity to mucosal cells.- - - - - - - - - - ranking = 0.65208850647544keywords = abdominal pain (Clic here for more details about this article) |
10/144. Primary angiitis of the central nervous system and Alzheimer's disease: clinically and pathologically evident in a single patient.Two years after being successfully treated for biopsy confirmed primary angiitis of the central nervous system (PACNS), a 69-year-old woman presented with cognitive decline. In contrast to her first presentation, her altered mental function developed gradually, was not associated with headache or abnormal cerebrospinal fluid analysis, and did not improve with immunosuppression. Reevaluation of her original brain biopsy not only confirmed the presence of PACNS, but also revealed neuritic plaques and neurofibrillary tangles, suggesting a concurrent diagnosis of Alzheimer's disease. Cerebral angiogram did not suggest vasculitis and magnetic resonance imaging showed generalized cerebral atrophy supporting the diagnosis of Alzheimer's. This case illustrates that Alzheimer's dementia and PACNS can coexist in a single patient and that Alzheimer's disease should be considered when a patient with successfully treated PACNS presents with cognitive decline months or years after initial diagnosis.- - - - - - - - - - ranking = 0.32429555543009keywords = headache (Clic here for more details about this article) |
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