1/314. Peripheral ulcerative keratitis--an extracutaneous neutrophilic disorder: report of a patient with rheumatoid arthritis, pustular vasculitis, pyoderma gangrenosum, and Sweet's syndrome with an excellent response to cyclosporine therapy.The term peripheral ulcerative keratitis represents a spectrum of inflammatory diseases, characterized by cellular infiltration, corneal thinning, and ulceration. Neutrophilic dermatoses are rarely associated with peripheral ulcerative keratitis. To date, peripheral ulcerative keratitis has only been reported in patients with pyoderma gangrenosum. Separate episodes of pyoderma gangrenosum, Sweet's syndrome, and pustular vasculitis developed in a 60-year-old patient with rheumatoid arthritis over an 8-year period. Over the past 2 years, 3 episodes of peripheral ulcerative keratitis occurred. cyclosporine (4 mg/kg/d) treatment was started on confirmation of pyoderma gangrenosum. Over the ensuing 2 years, it became evident that the activity of her ocular and skin diseases, as well as her arthritis, paralleled the administration or cessation of cyclosporine therapy. Dermatologists should be aware of the association of Sweet's syndrome, pyoderma gangrenosum, and pustular vasculitis with peripheral ulcerative keratitis. This rare ocular manifestation and the serious sequelae when left untreated make recognition crucial. cyclosporine proved to be a very effective treatment for all of our patient's diseases.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
2/314. poems syndrome, steroid-dependent diabetes mellitus, erythema elevatum diutinum, and rheumatoid arthritis as extramedullary manifestations of plasma cell dyscrasia.poems syndrome is a rare synopsis of different multisystemic disorders (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammapathy, and skin lesions) associated with plasma cell dyscrasia. We herein report the atypical case of a 44-year-old white man presenting with glomerulopathy, poems syndrome, and erythema elevatum diutinum with a few-year history of non-insulin-dependent diabetes mellitus (NIDDM) and seronegative rheumatoid arthritis (RA) as early manifestations of IgAlambda multiple myeloma. The prescription of 1 mg/kg/day prednisone improved the patient's features dramatically. Skin lesions improved by the association of glucocorticoids and plasma exchange, recurred when plasmapheresis ceased, and remitted when plasma exchange was reintroduced. NIDDM requiring insulinotherapy recurred when corticoids were discontinued and remitted when prednisone was reintroduced. However, prednisone and plasmapheresis had no effect on polyneuropathy, M-paraprotein, and plasma cell dyscrasia in our patient, who developed indolent multiple myeloma a few years later. We thus concluded that poems syndrome, steroid-dependent diabetes mellitus, rheumatoid arthritis, RA, and skin vasculitis in our patient were triggered by plasma cell dyscrasia.- - - - - - - - - - ranking = 1.1666666666667keywords = syndrome (Clic here for more details about this article) |
3/314. Remission of the nephrotic syndrome in a patient with renal amyloidosis due to rheumatoid arthritis treated with prednisolone and methotrexate.A 46-year-old woman developed nephrotic syndrome secondary to rheumatoid arthritis (RA). A renal biopsy showed deposition of amyloid fibrils in the subendothelial space of the glomerular capillary walls. After treatment with prednisolone (PSL, 40 mg/day), the levels of c-reactive protein (CRP) and serum amyloid A decreased to within normal limits for 2 weeks. However, the nephrotic syndrome persisted for 6 months after the therapy. To maintain the suppression of disease activity and to reduce PSL, methotrexate (5 mg/week) was added. The nephrotic syndrome resolved gradually, and the level of serum albumin returned to normal. Although renal prognosis of patients with nephrotic syndrome due to amyloidosis caused by RA has been considered poor, adequate and long-term treatment of RA with antiinflammatory drugs, including PSL and methotrexate, is useful for patients with secondary amyloidosis complicated by RA.- - - - - - - - - - ranking = 1.3333333333333keywords = syndrome (Clic here for more details about this article) |
4/314. methotrexate-induced papular eruption in patients with rheumatic diseases: a distinctive adverse cutaneous reaction produced by methotrexate in patients with collagen vascular diseases.BACKGROUND: In the past few years, low doses of methotrexate have been used for treatment of patients with rheumatoid arthritis and other collagen vascular diseases, mainly as an immunosuppressive and corticosteroid-sparing drug. Several cutaneous adverse reactions have been described in association with methotrexate therapy. OBJECTIVE: We describe the clinical and the histopathologic features of distinctive cutaneous lesions that appeared in 4 patients with acute bouts of collagen vascular diseases who were receiving methotrexate therapy. methods: We clinically and histopathologically evaluated cutaneous lesions caused by methotrexate therapy in 4 patients, 2 with systemic lupus erythematosus, 1 with rheumatoid arthritis, and 1 with Sharp syndrome. RESULTS: Clinically, lesions consisted of erythematous indurated papules most commonly located on proximal areas of the extremities. Histopathologic examination of these papules showed an inflammatory infiltrate mainly composed of histiocytes interstitially arranged between collagen bundles of the dermis, intermingled with few neutrophils. In some foci of deeper reticular dermis, small rosettes composed of clusters of histiocytes surrounding a thick central collagen bundle were seen. Cutaneous lesions showed a direct chronologic relationship with methotrexate therapy, and they disappeared when the drug was tapered or withdrawn and corticosteroids were increased. CONCLUSION: patients receiving low doses of methotrexate for acute bouts of collagen vascular diseases may experience characteristic cutaneous lesions with distinctive clinical and histopathologic findings shortly after methotrexate administration. We discuss the differential diagnosis with other dermatoses showing similar histopathologic findings that have been described in patients with collagen vascular diseases.- - - - - - - - - - ranking = 0.16666666666667keywords = syndrome (Clic here for more details about this article) |
5/314. Rheumatoid arthritis and multiple sclerosis in the same patient. Two case-reports.We report on two new patients with both rheumatoid arthritis and multiple sclerosis. In one patient, the first manifestations of multiple sclerosis occurred eight years after onset of seronegative rheumatoid arthritis without extraarticular manifestations. The other patient had a 20-year history of multiple sclerosis when she developed seropositive, nodular rheumatoid arthritis. Neither patient had evidence of systemic lupus erythematosus. A lip biopsy was done in one patient, with normal results; the other patient was free of clinical symptoms of sicca syndrome and had a negative Schirmer's test. The paucity of similar cases in the literature is surprising since multiple sclerosis and rheumatoid arthritis are both autoimmune diseases and share many pathophysiologic and etiologic features. Although chance alone may explain the occurrence of both conditions in the same patient, the existence of shared etiologic factors should in theory increase the likelihood of the association.- - - - - - - - - - ranking = 0.16666666666667keywords = syndrome (Clic here for more details about this article) |
6/314. An aggressive form of polyarticular arthritis in a man with CD154 mutation (X-linked hyper-IgM syndrome).Hyper-IgM syndrome (HIM) is a rare immunodeficiency disorder that has been associated with the development of symptoms and clinical features characteristic of rheumatoid arthritis (RA). We describe a patient with HIM and severe erosive arthritis with prominent nodules in the absence of detectable serum rheumatoid factor. Because HIM results from defects in either T cell CD154 (cd40 ligand) expression or abnormal CD40 signaling, the molecular basis of the patient's disease was analyzed. Activated CD4 T cells failed to express surface CD154 protein, and molecular analysis of CD154 complementary dna revealed a nucleotide transversion resulting in the nonconservative amino acid substitution G-D at amino acid 257. This case indicates that defective CD154-dependent CD40 signaling can be associated with susceptibility to a severe inflammatory arthritis that has both similarities to and differences from idiopathic RA.- - - - - - - - - - ranking = 0.83333333333333keywords = syndrome (Clic here for more details about this article) |
7/314. Neutrophilic lobular (pustular) panniculitis associated with rheumatoid arthritis: a case report and review of the literature.Rheumatoid nodules, which affect the subcutis around joints, are the most frequent specific cutaneous lesions of rheumatoid arthritis (RA). panniculitis is a rarely reported and nonspecific complication of RA. We report a 42-year-old woman with seropositive RA who presented with a 2-month history of lower leg panniculitis. biopsy of a leg nodule showed a lobular neutrophilic infiltrate with lipophages and central basophilic necrosis. In addition, focal changes of lipomembranous fat necrosis indicative of ischemic damage were identified at the margins of the lobular infiltrate. Neutrophilic lobular panniculitis is commonly detected in panniculitis secondary to bacterial infections, pancreatitis, and factitial causes. However, this pattern of panniculitis has also been reported in some cases of erythema nodosum-like lesions found in Behcet disease or bowel bypass syndrome and in rare cases of seropositive RA. These reported histologic findings fall into the spectrum of neutrophilic vascular reactions described by Jorizzo and Daniels for RA-associated dermatoses. In view of these findings. RA and related neutrophilic dermatoses (e.g., Behcet disease) should be included in the differential diagnosis of neutrophilic lobular panniculitis.- - - - - - - - - - ranking = 0.16666666666667keywords = syndrome (Clic here for more details about this article) |
8/314. Cystic rheumatoid arthritis with Felty's syndrome and ankylosing spondylitis.A 63-year-old man with strictly axial ankylosing spondylitis since the age of 28 years had a seven-year history of cystic seronegative rheumatoid arthritis with Felty's syndrome. cysts were present in the hands, feet, wrists, shoulders, hips, one elbow, and one knee. There was no evidence of juxtaarticular demineralization, joint space loss, erosions, or joint destruction. Rheumatoid pannus was demonstrated within the cysts, particularly at the hip, ruling out cystic hip disease due to ankylosing spondylitis. HLA typing demonstrated the B27 and DR4 haplotypes. HLA B27 may be associated with a worse prognosis of rheumatoid hip involvement.- - - - - - - - - - ranking = 0.83333333333333keywords = syndrome (Clic here for more details about this article) |
9/314. Rheumatoid arthritis associated with myelodysplastic syndrome: a case report.myelodysplastic syndromes (MDS) are a group of refractory anemias resulting from a clonal stem cell disorder often associated with cytogenetic abnormalities. There is increasing recognition of immunological abnormalities in patients with MDS, including defective B- and T-cell function, hyper- or hypogammaglobulinemia and monoclonal gammopathy. MDS have been associated with sjogren's syndrome, polymyalgia rheumatica, relapsing polychondritis and systemic lupus erythematosus. Although there may be various rheumatologic features, including acute arthritis in MDS, chronic inflammatory arthritis is uncommonly combined. There have been a few reports that described cases of rheumatoid arthritis (RA) concurrent with MDS, but advanced rheumatoid arthritis with typical joint deformities has rarely been reported. We report a case of rheumatoid arthritis with atlantoaxial subluxation combined with refractory anemia in a 31-year-old woman.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
10/314. Acute anterior forearm compartment syndrome following wrist arthrodesis.The authors report a case of anterior compartment syndrome in the forearm following total wrist arthrodesis via a dorsal approach. As far as they know this is the second case reported.- - - - - - - - - - ranking = 0.83333333333333keywords = syndrome (Clic here for more details about this article) |
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