1/5. Successful treatment of B cell chronic lymphocytic leukemia-associated severe paraneoplastic pemphigus with cyclosporin A.Since the first description of paraneoplastic pemphigus, several cases have been described in the literature. However, curative therapy is usually a challenge to the physicians treating this disease. Several publications are available discussing the efficacy of steroids, cyclophosphamide and cyclosporin A. Recently, a report of the successful use of rituximab was also published. However, the use of cyclosporin A is controversial in the case of B cell malignancies, as there are reports showing the cytotoxic effect of this drug on B cells. However, other authors report no effect, or even unwanted effects resulting in B cell proliferation. We report the case of a 50-year-old Caucasian male. He developed a B cell lymphoma consisting of CD5/CD20-double-positive cells, and 2 months later, it was followed by a very severe paraneoplastic pemphigus affecting the mucosa and the skin. The lymphoma was well managed with CHOP and CVP polychemotherapy, followed by oral chlorambucil; however, the bullous eruptions did not disappear. Oral steroids, cyclophosphamide, plasmapheresis and IVIG therapy were only partially successful, so we decided to use oral cyclosporin A. Starting with 7 mg/kg and maintaining a steady plasma level of no less then 110 ng/l, the bullae completely disappeared within 6 weeks, and the patient has been in remission for 17 months now, taking the oral cyclosporin A continuously. The underlying B cell disorder did not relapse during the therapy.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
2/5. pemphigus vulgaris: an acquired blistering disease.pemphigus vulgaris is one of a group of autoimmune disorders that are caused by autoantibodies against the desmoglein adhesion molecules of squamous epithelial cells. It is a rare form of immune dysfunction that can prove vexing to the patient and physician, but it has distinct clinical and histologic findings. We report a case of a patient with the autoimmune blistering disease pemphigus vulgaris localized to the oral cavity and discuss the important clinical, immunopathologic, and therapeutic factors of this disease. This case report highlights the unusual nature of pemphigus vulgaris, the modalities used in its diagnosis, and effectiveness of therapy. pemphigus vulgaris is an uncommon disease blistering disorder due to desmoglein autoantibodies, whose presentation can be alarming and puzzling to the clinician. awareness of the disease's presentation and mechanism will allow for an efficient diagnostic evaluation and timely treatment.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
3/5. Immune complex disease of the skin.The physician can now recognize clinically and histopathologically the cutaneous manifestations of immune complex disease. The usual clinical environment in which this type of reaction occurs has been very specifically delineated. Studies of immunoglobulins, complement components, and B cells in the blood may confirm the nature of the reaction. Special studies of cryoproteins of C1q precipitin or radioimmunoassay procedures may demonstrate directly the complexes in the blood. biopsy of skin for immunofluorescence is confirmative of the skin disease and the presence of immune complexes. biopsy of normal skin may be prognostic and indicate severity of the disease. Lesions may be induced by epinephrine, trauma, and controlled imflammation for clinical and pathologic study and confirmation of diagnosis. Treatment of the disease with corticosteroids, sulfapyridine, nicotinic acid, and antimalarial drugs may be useful. clofazimine is an intriguing experimental drug. Plasmaphoresis has worked well with some patients.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
4/5. pemphigus following hepatitis B vaccination--coincidence or causality?pemphigus is an autoimmune blistering disease caused by autoantibodies against epithelial intercellular components. Its etiology is unknown, and neoplasms, antecedent infections or medications are considered possible triggering factors for the disease in some cases. We describe the first case of pemphigus following a hepatitis b virus vaccination. We suggest that in some cases vaccination may be the triggering factor for pemphigus in genetically predisposed individuals and physicians should be aware of this possible association.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
5/5. Prepubertal oral pemphigus vulgaris.Although rare, oral pemphigus vulgaris must be considered in those patients who have a prolonged history of oral lesions. The fact that chronic oral lesions characteristically precede skin changes in pemphigus emphasizes the importance of this disease to the dental profession. patients with long-term and recurrent vesiculoulcerative oral lesions should undergo biopsy examination to establish the diagnosis. The dentist and the physician must collaborate to establish an appropriate diagnostic and therapeutic plan for the management of these patients.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |