1/7. Paraneoplastic pemphigus in association with hepatocellular carcinoma.Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous blistering disease associated with neoplasms, most frequently of the lymphoproliferative type. Rare PNP cases related to nonhematological solid tumors have been reported. The patient in this report presented with severe mucocutaneous involvement of PNP associated with hepatocellular carcinoma. Histopathology showed vacuolar interface dermatitis with keratinocyte necrosis and intraepidermal acantholysis. Direct immunofluorescence exhibited deposition of intercellular IgG and complement and granular complement at the dermoepidermal junction. Indirect immunofluorescence testing showed a typical intercellular staining on monkey esophagus and rat bladder epithelium. immunoprecipitation showed characteristic target antigens of 250, 210, and 190 kDa molecular weights. This patient met all diagnostic criteria for paraneoplastic pemphigus and is, to our knowledge, the first report of a case associated with hepatocellular carcinoma.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/7. Fatal case of community-acquired pneumonia caused by legionella longbeachae in a patient with systemic lupus erythematosus.Reported here is a rare case of atypical pneumonia due to a non- pneumophila Legionella sp. that occurred in a young patient with systemic lupus erythematosus. In spite of aggressive treatment, the patient died 24 h following admission to the intensive care unit. legionella longbeachae was cultured from respiratory tract specimens and identified to the genus level by PCR and to the species level by an immunofluorescence test. Since most current laboratory tests for Legionella spp., including urinary antigen and serology, cannot detect infections caused by non- pneumophila Legionella spp., culture on legionella-selective media should be strongly considered when diagnosing immunosuppressed patients with pneumonia.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/7. A rare case of intravascular coagulation after honey bee sting.A patient presented with coagulation problems a few days after honeybee sting. The purpuric skin changes developed on the legs and buttocks. She manifested signs of hypotension with disturbance of consciousness. Allergen-specific IgE serum levels against honey bee venom antigens reached >17.5 kU/l. The platelet count was 33,000/ml . The prothrombin index decreased to 28%, prothrombine time was prolonged to 34". fibrin degradation products were present in serum. After 10 day treatment the girl improved, but necrotic skin changes required further plastic surgery. Honeybee sting problems should be taken into account as a cause of coagulation problems.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/7. Lymphangiomyomatosis arising in the pelvic cavity: a case report.lymphangioleiomyomatosis (LAM) is a rare disease usually occurring in young women of child-bearing age. It is characterized by a distinctive proliferation of lymphatic smooth muscle cells, especially occurring in the pulmonary parenchyme. The majority of primary LAM occurs in the lung, but there are a few reports of extrapulmonary cases. We report a case of a 21-yr-old female who first complained of low abdominal pain and was referred from a local clinic with the impression of an ovarian cyst. Gynecologic ultrasonography revealed a large posterior pelvic mass with an irregular echogenicity measuring 9.7 x 4.2 cm in size. Pelviscopy showed a large, thin walled, partly cystic, pelvic mass. The mass was partly removed. Microscopically, the mass was characterized by a haphazard proliferation of smooth muscle cells arranged in fascicular, trabecular, and papillary patterns around a ramifying network of endothelium-lined spaces. The cells were plump or epithelioid with abundant eosinophilic cytoplasm and showed a positive reaction for both alpha-smooth muscle actin and HMB-45 antigen. Surgical and pathological findings were consistent with pelvic retroperitoneal LAM. Despite the numerous treatment attempts, the patient suffered from intractable chylous ascites and developed pulmonary LAM and died due to severe respiratory distress.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/7. Takayasu's arteritis and ulcerative colitis in a young non-Asian woman: a rare association.The association between ulcerative colitis and Takayasu's arteritis has been well described in patients of Asian ethnicity. We present the third non-Asian case reported in the English literature, of a 37-year-old woman with a 4-year history of Takayasu's arteritis, who developed idiopathic ulcerative colitis. She was found to carry the human leucocyte antigens HLA-B52 and DR2, which have been previously noted to be associated with these inflammatory conditions, mainly in the Japanese population. Ulcerative colitis was steroid-dependent despite simultaneous administration of mesalazine. Azathioprime achieved remission of both diseases. The possible pathogenic association of the disorders is discussed.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/7. Merkel cell carcinoma -- a rarity in the urogenital tract.BACKGROUND: Merkel cell carcinoma -- a rare, aggressive cancer of the skin integument - is being increasingly diagnosed but represents an absolute rarity in the urogenital tract. CASE REPORT: We report on a 70-year-old man who was referred to us with suspected testicular cancer. The pathology report revealed a metastasized Merkel cell carcinoma. Fulminant disease progression under chemo-therapy (regimen as for small cell lung cancer) resulted in death 5 months later. CONCLUSION: The patient described is considered to be the first to develop testicular metastasis derived from Merkel cell carcinoma. Besides neuroendocrine and epithelial antigen tests, somatostatin receptor scintigraphy is a helpful diagnostic tool. New receptor-associated therapies may allow more effective and less toxic treatment modalities in the mostly elderly or immune deficient patients.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/7. Post-transfusion purpura: a challenging diagnosis.BACKGROUND: Post-transfusion purpura is a rare syndrome characterized by severe thrombocytopenia and bleeding caused by alloimunization to human platelet specific antigens following a blood component transfusion. The suggested incidence is 1:50,000-100,000 transfusions, most often occurring in multiparous women. The diagnosis is not easy because these patients, who are often critically ill or post-surgery, have alternative explanations for thrombocytopenia such as infection, drugs, etc. OBJECTIVES: To describe patients with initially misdiagnosed PTP and to emphasize the diagnostic pitfalls of this disorder. patients AND RESULTS: During a period of 11 years we diagnosed six patients with PTP, four women and two men. The incidence of PTP was approximately 1:24,000 blood components transfused. We present the detailed clinical course of three of the six patients in whom the diagnosis was particularly challenging. The patients were initially misdiagnosed as having heparin-induced thrombocytopenia, systemic lupus erythematosus complicated by autoimmune thrombocytopenia, and disseminated intravascular coagulation. A history of recent blood transfusion raised the suspicion of PTP and the diagnosis was confirmed by appropriate laboratory workup. CONCLUSIONS: PTP seems to be more frequent than previously described. The diagnosis should be considered in the evaluation of life-threatening thrombocytopenia in both men and women with a recent history of blood transfusion.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |