1/9. Effect of myocutaneous inflammatory changes caused by intra-arterial chemotherapy on the outcome of patients who undergo limb-saving surgery.BACKGROUND: This study examined the effect of myocutaneous inflammatory changes caused by intra-arterial chemotherapy on the outcome of patients who undergo limb-saving surgery. methods: One hundred seven patients with malignant bone and soft tissue tumors were administered intra-arterial cisplatin and caffeine preoperatively with or without doxorubicin. Nine patients (8.4%) who had had myocutaneous inflammatory reactions were reviewed to determine the effects of this complication on limb-saving surgery. RESULTS: The patients complained of unbearable and continuous pain while undergoing intra-arterial infusion. gait disturbances and muscle weakness were caused by their severe regional pain, resulting in the use of crutches even before surgery. Extended areas of necrotic skin and muscle were resected simultaneously with limb-saving surgery on four patients. Myocutaneous necrosis in one patient, indurations in two patients, and flares in two patients were not treated surgically. pain relief was provided to eight patients at some point. Four patients with extensive myocutaneous necrosis around the knee joint had restricted range of motion and poor functional results. radionuclide angiography using 99m-technetium-macroaggregated albumins was performed to evaluate the blood flow to the affected muscle and tumor. It showed marked increase of the radioisotope perfusion in the affected muscles but little uptake in the tumor. These results may suggest that the affected muscles diminish the effects of anticancer drugs on the tumors. CONCLUSIONS: Myocutaneous inflammatory reactions should be prevented if possible to obtain good limb function and chemotherapeutic effects in patients who undergo intra-arterial chemotherapy. Resection of necrotic tissue is mandatory to relieve pain.- - - - - - - - - - ranking = 1keywords = soft (Clic here for more details about this article) |
2/9. An ultrastructural study of pigmented purpuric dermatitis with special reference to fibrous long-spacing collagen.A case of pigmented purpuric dermatitis (PPD) in a Japanese man aged 59 years is reported with an interesting ultrastructural finding. Clinically, the lesions, which consisted of telangiectatic puncta and pigmentation, were irregular in shape and occurred predominantly on the lower legs without pruritus. Histologically, lymphocytic perivascular infiltrates and extravasation of red blood cells were observed in the papillary dermis. Ultrastructurally, endothelial cells with ovoid nuclei showed swelling and the lumen of the capillary became narrowed. Several banded structures, so-called fibrous long-spacing collagen (FLSC), were observed in the cytoplasm. They were spindle shaped, about 5 microm in length, and showed crossbands of 300-nm-wide intervals with fine intraperiodic bands. These structures were not observed in dermal connective tissue and fibroblasts. These results suggested that FLSC was synthesized in endothelial cells rather than being phagocytosed by endothelial cells, which might be helpful in investigating the etiology of PPD.- - - - - - - - - - ranking = 119.06507709571keywords = connective (Clic here for more details about this article) |
3/9. Palisaded neutrophilic granulomatous dermatitis in rheumatoid arthritis.Palisaded neutrophilic granulomatous dermatitis (PNGD) is an entity that has not been clearly defined either clinically or histopathologically. It is seen in patients with rheumatoid arthritis and other connective tissue diseases. In the past, many cases of PNGD have been described under several different names including palisaded neutrophilic and granulomatous dermatitis, linear subcutaneous bands, interstitial granulomatous dermatitis with cutaneous cords and arthritis, rheumatoid papules, and Churg-Strauss granuloma. We report 7 additional cases of PNGD. Clinically, 6 patients presented with erythematous to violaceous plaques, papules, and nodules on multiple body sites; one presented with subcutaneous linear bands on the shoulder. Five had rheumatoid arthritis; one had adult-onset Still's disease; and one showed clinical signs of rheumatoid arthritis, although serologically the rheumatoid factor was negative. On histologic examination, a spectrum of changes was observed ranging from urticaria-like infiltrates to leukocytoclastic vasculitis and granuloma annulare with neutrophils. We report these cases to expand the histologic spectrum of this entity and to further delineate the different forms of clinical presentation.- - - - - - - - - - ranking = 119.06507709571keywords = connective (Clic here for more details about this article) |
4/9. Palisaded neutrophilic granulomatous dermatitis presenting as an unusual skin manifestation in a patient with Behcet's disease.A 32-year-old Korean woman with painful oral ulcers and a sore throat presented with multiple erythematosus papules on both legs. Histological examination of the papular lesions on the legs demonstrated palisaded granuloma with degeneration of collagen fibres in the dermis, compatible with palisaded neutrophilic granulomatous dermatitis (PNGD). This condition is known to be an unusual disease entity associated with various systemic autoimmune diseases, such as rheumatoid arthritis, systemic lupus erythematosus, other connective tissue diseases, and systemic vasculitis. To our knowledge a case with typical Behcet's disease coinciding with PNGD among systemic autoimmune diseases has not been described before.- - - - - - - - - - ranking = 119.06507709571keywords = connective (Clic here for more details about this article) |
5/9. Inflammatory destruction of elastic fibers in acquired cutis laxa is associated with missense alleles in the elastin and fibulin-5 genes.cutis laxa (CL) is a condition characterized by redundant, pendulous, and inelastic skin. Acquired CL has been reported in patients with inflammatory diseases. The goal of this study was to investigate whether genetic lesions predispose patients to the development of acquired CL. We report a patient who developed CL following a toxocara canis parasitism. He later had an aortic root aneurysm that required surgical correction. Histological evaluation showed inflammation followed by destruction of elastic fibers in both the skin and the aorta. Mutational analysis showed that the patient was heterozygous for an inherited fibulin-5 (FBLN5) allele G202R and compound heterozygous for elastin (ELN) alleles A55V and G773D. Western blotting indicated abnormal proteolytic processing of tropoelastin (TE) in patient fibroblasts. The FBLN5 202R allele on the other hand led to increased interaction of FBLN5 and TE and increased deposition of insoluble ELN partially rescuing the deficiency conferred by ELN mutation G773D. We demonstrated that the interaction of ELN and FBLN5 alleles results in elastic fibers susceptible to inflammatory destruction. These results suggest that the pathogenesis of acquired CL involves an underlying genetic susceptibility and highlight the importance of molecular genetic analysis in patients with idiopathic connective tissue disorders.- - - - - - - - - - ranking = 119.06507709571keywords = connective (Clic here for more details about this article) |
6/9. Pseudoepitheliomatous hyperplasia and pyoderma gangrenosum after a brown recluse spider bite.brown recluse spider bites may result in extensive soft tissue injury, causing months of disability. We have described a patient who had a series of extensive surgical debridements after envenomation. Despite skin grafting, persistent cutaneous lesions and extensive satellitosis progressed to involve the entire lower extremity. A recent biopsy showed pseudoepitheliomatous hyperplasia and pyoderma gangrenosum complicating the original injury. Although the role of early surgical excision and newer forms of medical treatment including dapsone and antivenom are still in evolution, recent reports suggest that the majority of patients will respond to medical therapy and may not require any surgical intervention.- - - - - - - - - - ranking = 1keywords = soft (Clic here for more details about this article) |
7/9. Unusual case of diffuse connective tissue disease with nodule formations in muscle, lung, kidney and brain.A 9-year-old boy had nodular masses in the left gastrocnemius muscle and lung, right kidney and right frontal area of the brain. Laboratory examinations, including positive anti-nuclear antibody, anti-RNP antibody, RA and CRP, hyper-gamma-globulinemia and accelerated ESR, showed characteristic of diffuse connective tissue disease (DCTD). biopsy specimens were obtained from the thymus and from masses in the left lung and gastrocnemius muscle. The thymus showed hyperplasia, and a mass in the lung showed nonspecific inflammatory reactions such as connective tissue proliferation and cellular infiltration. Biopsied muscle also showed severe connective tissue proliferation, cellular infiltration, variation in fiber size and thickened blood vessel walls. In addition, abnormalities, including thickening of the basement membrane and an almost occluded narrow lumen of capillaries, were found on electron microscopy examination. Steroid administration led to remarkable improvement of the symptoms. These results suggested that DCTD is responsible for these symptoms.- - - - - - - - - - ranking = 833.45553966997keywords = connective (Clic here for more details about this article) |
8/9. head and neck manifestations of dermatomyositis-polymyositis.dermatomyositis and polymyositis are rare disorders of connective tissue that manifest themselves primarily as muscular weakness. Fifty-five percent of 45 patients with this diagnosis at Thomas Jefferson University Hospital had symptoms referable to the head and neck. While dysphagia and the cutaneous manifestation predominate, such symptoms as facial swelling and oral mucosal disorders may require the treating physician to seek the advice of an otolaryngologist before the patient suffers any muscular changes. A thorough knowledge of this disease entity is essential to specialists in head and neck diseases.- - - - - - - - - - ranking = 119.06507709571keywords = connective (Clic here for more details about this article) |
9/9. Treatment of equinocavovarus deformity in adults with the use of a hinged distraction apparatus.We evaluated the results following the use of the Volkov-Oganesyan-Povarov hinged distraction apparatus to correct equinocavovarus deformity of the foot in sixty-five adults (eighty-three feet). The deformity was secondary to ischemic and neuropathic changes after trauma to the foot and ankle, poliomyelitis, charcot-marie-tooth disease, or untreated clubfoot. The deformity often was associated with severe neurotrophic changes, extensive scarring of the skin, callosities, or osteomyelitis of the foot, all of which usually preclude operative reconstruction. The average age of the patients was twenty-six years (range, sixteen to fifty-six years). The average duration of follow-up was ten years (range, three to nineteen years). The duration of distraction was three to thirteen weeks, and the apparatus usually was kept in place for an additional two months after the desired position of correction had been achieved. The patient then wore a plaster cast for an average of eight weeks and custom-molded shoe-inserts for at least one year. A plantigrade foot was obtained with this external transosseous distraction device. Eight patients had inflammation of pin tracks, which was treated with local injection of antibiotics into the soft tissues and application of bandages soaked in antibiotics to the area around the pin sites. The pins were removed from five of the eight patients, and the apparatus was removed from three additional patients because of osteomyelitis. The results were classified as good in fifty-nine feet, satisfactory in twenty, and unsatisfactory in four. In the four feet (three patients) with an unsatisfactory result, a good result was obtained after an arthrodesis was done.- - - - - - - - - - ranking = 1keywords = soft (Clic here for more details about this article) |