1/344. Anticoagulant-induced skin necrosis in a patient with hereditary deficiency of protein s.Skin necrosis is a rare but debilitating complication of treatment with vitamin k antagonist anticoagulants such as warfarin. A clinically similar syndrome has been reported less frequently with heparin therapy. We recently managed a thirty-year-old female patient who developed skin necrosis on her left lower extremity while on warfarin for postpartum DVT. The lesions started to develop 48 hr after stopping heparin therapy. Discontinuation of warfarin and reinstitution of heparin was complicated by a rapid decrease in platelet count consistent with heparin-induced thrombocytopenia (HIT) and its associated risk of platelet activation and thrombosis. The diagnosis was supported by the identification of antibodies against heparin/platelet factor 4 complexes in the patient's serum. The platelet count recovered and the patient improved after switching to therapy with the heparinoid danaparoid. Evaluation for a hypercoagulable state revealed a partial deficiency of protein s, a condition that previously was identified in two of her family members. It is not clear if this patient suffered from warfarin-induced skin necrosis, a manifestation of heparin-mediated platelet activation, or a complex condition in which both drugs contributed. HIT may affect 1-3% of patients who receive unfractionated heparin, and this case raises the possibility that heparin may contribute to, or cause, some episodes of skin necrosis attributed to warfarin. Because many patients who develop warfarin-induced skin necrosis have been treated initially with heparin, it would seem prudent to consider HIT in these situations.- - - - - - - - - - ranking = 1keywords = family member, member (Clic here for more details about this article) |
2/344. Cell-mediated immunity in epidermodysplasia verruciformis.Investigations were performed in 6 cases of epidermodysplasia verruciformis and 2 healthy family members. Nonspecific cell-mediated immunity (CMI) was studied by measuring response to phytohemagglutinin (PHA) and concanavalin a (Con A), percentrages of E- and EAC-rosette-forming lymphocytes, bacterial skin tests, and allergic reactions to dinitrochloro-benzene (DNCB). Impairment of CMI was manifested by reduction in the percentage of E rosettes, and lowered response to PHA, and- to a lesser degree- to Con A. The immune response to DNCB sensitization was invariably negative. Impairment of CMI was greater in cases of long duration and with extensive lesions. The cases of similar duration and extent of lesions, which never showed tendency to tumor formation, were not different in CMI in comparison with cases with numerous tumors. Only in cases with very advanced tumors CMI was impaired parallel to the gravity of the patient's general condition.- - - - - - - - - - ranking = 1keywords = family member, member (Clic here for more details about this article) |
3/344. Primary subcutaneous nocardial infection in a SLE patient.A patient with systemic lupus erythematosus (SLE) developed primary subcutaneous nocardiosis during steroid and cyclophosphamide therapy for diffuse proliferative glomerulonephritis. In spite of local process the patient manifested signs of general deterioration mimicking SLE exacerbation. The diagnosis was made by bacteriologic examination of the material obtained by CT guided aspiration. Surgical drainage and systemic treatment with trimethoprim/sulphamethoxazole (TMT/SMZ) 960 mg twice/d led to a clinical recovery and enabled the continuation of the steroid and cytotoxic regimen.- - - - - - - - - - ranking = 0.014825097467338keywords = life (Clic here for more details about this article) |
4/344. Cutaneous waldenstrom macroglobulinemia in transformation.waldenstrom macroglobulinemia is a low-grade B-cell lymphoproliferative disorder of the elderly with characteristic monoclonal IgM-producing neoplastic infiltrates of the bone marrow, lymph node, and spleen. Cutaneous manifestations are usually nonspecific such as purpura, ulcers, and urticarial lesions. These lesions are caused by hyperviscosity of the blood, immune complex-mediated vascular damage, paraprotein deposition, and amyloid deposition. Specific skin lesions occur rarely and generally consist of translucent, flesh-colored papules composed of monoclonal IgM deposits. Rarely, there may be violaceous lesions composed of low-grade lymphoplasmacytic infiltrates characteristic of waldenstrom macroglobulinemia. Both cutaneous manifestations of the disease, as well as disease transformation to high-grade, large cell lymphoma are rare. We report two very unusual cases of waldenstrom macroglobulinemia with documented skin disease that demonstrated transformation to high-grade lymphoma. Both patients were elderly men with long-standing waldenstrom macroglobulinemia involving the bone marrow, who subsequently developed skin involvement by the disease. waldenstrom macroglobulinemia can rarely manifest as cutaneous disease, sometimes as a high-grade transformation of low-grade waldenstrom macroglobulinemia elsewhere. Distinction of cases of transformed waldenstrom macroglobulinemia from de novo cutaneous large cell lymphoma may be important, because the two entities are likely biologically different.- - - - - - - - - - ranking = 0.014825097467338keywords = life (Clic here for more details about this article) |
5/344. sweat gland proliferations in scleromyxedema.Eccrine sweat duct proliferations may be found in various inflammatory and neoplastic skin lesions. We report a patient with scleromyxedema with extensive proliferations of intradermal sweat ducts. Three-dimensional reconstruction demonstrated extensive coiling and branching of the sweat ducts leading into cystic lacunae. In contrast to the basal cell carcinoma that had grown within the scleromyxedematous skin, the ducts close to the lumen stained positive for carcinoembryonic antigen and could therefore be differentiated from basal cell carcinoma. In micrographically controlled surgery of cutaneous epithelial tumors that are located in chronically inflamed skin, such sweat gland proliferations have to be considered as differential diagnosis.- - - - - - - - - - ranking = 0.10377568227137keywords = life (Clic here for more details about this article) |
6/344. Epstein Barr virus-associated lymphoproliferative-disorders primarily involving the skin.In cases of solid organ or bone marrow transplantation, up to 2 to 10% of patients may develop lymphoproliferative disorders (LPD), often induced by Epstein-Barr virus (EBV). Despite a morphology mimicking malignant lymphoma, in some cases the lesions will disappear completely after the degree of immunosuppression is lowered. Lately, similar processes have been described in non-transplant, immunosuppressed patients. A SNOMED search was performed on the database of three hospitals between 1990 and 1997, to identify patients with immunosuppression-related lymphoproliferative disorders (IR-LPD) involving primarily the skin. Two patients were identified. One was 2 years after kidney transplantation, and the other was being treated with methotrexate for dermatomyositis. In both biopsies, there was a diffuse perivascular proliferation of large lymphocytes with ample cytoplasm and pleomorphic nuclei, associated with extensive dermal and subcutaneous necrosis. Immunohistochemical studies revealed expression of CD20, CD45RO, CD43, CD30, EBV-LMP1, and EBV-NA2 by the atypical lymphocytes in both cases and, in one case, of the EBV-transcriptional replication activation protein. In both cases the lesions completely disappeared and have not recurred. Primary involvement of the skin by IR-LPD is very rare. Based on our results, it is possible that some of these cases in the skin contain EBV and co-express CD30 and T- and B-cell markers. The diagnosis of IR-LPD should be considered in cutaneous lymphoid proliferations in immunosuppressed patients. Before rendering an unequivocal diagnosis of malignant lymphoma, reduction of immunosuppression and follow-up of 4-8 weeks should be considered.- - - - - - - - - - ranking = 0.11860077973871keywords = life (Clic here for more details about this article) |
7/344. The role of trisomy 8 in the pathogenesis of chronic eosinophilic leukemia.A case of chronic eosinophilic leukemia (CEL) manifesting as spinal cord compression by an extradural eosinophilic chloroma in a 32-year-old Chinese man was presented, who subsequently developed extramedullary transformation at the skin and then peritoneal cavity. Cytogenetic study of bone marrow cells at diagnosis showed a clonal karyotypic abnormality of trisomy 8 ( 8), which on fluorescence in situ hybridization (FISH) was shown to be present in a clone of abnormal eosinophils, hence showing the neoplastic nature of the eosinophilic proliferation. There was another population of abnormal eosinophils that did not show 8. At blastic transformation, all blast cells in ascitic fluid were shown by FISH to harbor 8. These findings suggest that 8 in this case may have arisen from clonal evolution and is not the primary genetic event in leukemogenesis, but 8 most probably imparts a further survival advantage to the clone responsible for subsequent blastic transformation.- - - - - - - - - - ranking = 0.014825097467338keywords = life (Clic here for more details about this article) |
8/344. Histopathological and cellular studies of a case of cutaneous radiation syndrome after accidental chronic exposure to a cesium source.This study was designed for the histopathological, cellular and biochemical characterization of a skin lesion removed surgically from a young male several months after accidental exposure to cesium-137, with an emphasis on expression of transforming growth factor beta1 (TGFB1) and tumor necrosis factor alpha (TNFA) and the occurrence of apoptosis. Under a hypertrophic epidermis, a highly inhomogeneous inflammatory dermis was observed, together with fibroblastic proliferation in necrotic areas. Immunostaining revealed overexpression of TGFB1 and TNFA inside the keratinocytes of the hypertrophic epidermis as well as in the cytoplasm of the fibroblasts and connective tissue of the mixed fibrotic and necrotic dermis. Inside this dermis, the TUNEL assay revealed areas containing numerous apoptotic fibroblasts next to areas of normal viable cells. Overexpression of TGFB1 was found in the conditioned medium and cellular fractions of both hypertrophic keratinocytes and fibrotic fibroblasts. This overexpression lasted for at least three passages in tissue culture. The present observations were consistent with the central role of TGFB1 in the determination of chronic radiation-induced damage to the skin and a significant involvement of TNFA. In addition, programmed cell death appeared to take place during the remodeling of the mixed fibrotic and necrotic tissue.- - - - - - - - - - ranking = 0.014825097467338keywords = life (Clic here for more details about this article) |
9/344. Proliferative verrucous leukoplakia with cutaneous involvement.Proliferative verrucous leukoplakia (PVL) is a distinct variant of oral leukoplakia characterized by a high rate of malignant transformation. Histologic features are variable and range from epithelial dysplasia to verrucous squamous cell carcinoma. To our knowledge, there have been no previous reports of cutaneous PVL. We present an interesting case of PVL involving the skin.- - - - - - - - - - ranking = 0.074125487336691keywords = life (Clic here for more details about this article) |
10/344. muir-torre syndrome: case report of a patient with concurrent jejunal and ureteral cancer and a review of the literature.BACKGROUND: muir-torre syndrome is a rare autosomal dominant genodermatosis, first described in 1967, characterized by the presence of sebaceous tumors and an internal malignancy in the absence of other predisposing factors. OBJECTIVE: Our purpose was to review and update published literature on muir-torre syndrome. methods: We describe a 66-year-old white man with a history of sebaceous tumors and newly diagnosed transitional cell cancer of the right ureter and adenocarcinoma of the jejunum. The literature on muir-torre syndrome is reviewed by means of medline search and available published reports and updated. RESULTS: Only 205 cases of muir-torre syndrome with 399 internal malignancies have been reported. The common presentation is the presence of sebaceous tumors along with a low-grade visceral malignancy. Sebaceous tumors appeared before the internal malignancy in 45 cases (22%), concurrently in 12 (6%), and after the internal malignancy in 114 (56%). In 33 (16%) of 205 patients, a temporal relationship was not reported. The total number of sebaceous gland carcinomas reported is 44; 17 of 44 were neoplasms of the meibomian gland. Keratoacanthomas have been noted in 48 (23%) of 205 patients. Gastrointestinal cancers are the most common internal malignancies (61%), followed by genitourinary (22%). CONCLUSION: The presence of sebaceous tumors warrants a search for an internal malignancy. In patients with muir-torre syndrome, regular follow-up and search for new malignancy is mandatory. Evaluation and monitoring of the family members of patients are also necessary. patients and their families should be counseled for genetic testing. Genetic analysis of the primary tumor and skin lesions should be arranged as an added research tool if possible to better understand the disease.- - - - - - - - - - ranking = 1keywords = family member, member (Clic here for more details about this article) |
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