1/30. Immunoablative high-dose cyclophosphamide without stem cell rescue in paraneoplastic pemphigus: report of a case and review of this new therapy for severe autoimmune disease.Paraneoplastic pemphigus (PNP) is a refractory and life-threatening autoimmune mucocutaneous disease. We have recently reported the effectiveness and safety of ablative intravenous cyclophosphamide (200 mg/kg daily over 4 days) without stem cell rescue in patients with refractory autoimmune diseases including systemic lupus erythematosus, autoimmune cytopenias, chronic inflammatory demyelinating polyneuropathy, and aplastic anemia. We report chronic lymphocytic leukemia-associated PNP in a patient who presented with extensive and debilitating painful oral ulcerations and received ablative therapy. The patient tolerated the regimen well and showed a slow but sustained improvement despite persistence of the underlying neoplasm. Eighteen months after therapy, the oral ulcerations were almost completely healed and the circulating autoantibodies became negative. Currently, the patient remains on cyclosporine and a low dose of prednisone. This provides further evidence for the efficacy and safety of this regimen in the management of severe autoimmune diseases including PNP.- - - - - - - - - - ranking = 1keywords = oral ulcer (Clic here for more details about this article) |
2/30. Paraneoplastic pemphigus: a case of long-term survival associated with systemic lupus erythematosus and polymyositis.A 35-year-old man presented with a generalized bullous eruption and oral ulceration. He had been diagnosed as having systemic lupus erythematosus and pelvic Castleman disease (hyaline-vascular type) in the past. Histologic, direct and indirect immunofluorescence and immunoprecipitation studies confirmed a diagnosis of paraneoplastic pemphigus (PNP). Initially several medical treatments were tried unsuccessfully. The pelvic tumor was surgically removed and the mucocutaneous lesions slowly regressed. Four years after presentation, he developed polymyositis which was completely controlled with short courses of corticosteroids. There was no evidence of relapse of PNP or lupus erythematosus at that time. At the 6-year follow-up he showed no clinical evidence of PNP, lupus erythematosus or polymyositis without requiring immunosuppressive therapy. This case emphasizes the fact that patients with benign-neoplasm-associated PNP may undergo complete remission of the autoimmune disease upon complete excision of the tumor. This case also stresses the possible association of PNP with other autoimmune diseases such as lupus erythematosus and polymyositis.- - - - - - - - - - ranking = 0.5keywords = oral ulcer (Clic here for more details about this article) |
3/30. Chronic oral mucosal ulceration in a 54-year-old female.pemphigus vulgaris most often begins in the mouth but is often overlooked in the differential diagnosis of chronic, multiple oral ulcerations and erosions. Accurate diagnosis requires perilesional biopsy including intact epithelium, submitted for hematoxylin and eosin as well as direct immunofluorescence staining. Early and aggressive treatment with moderate to high dose prednisone in combination with steroid sparing drugs such as azathioprine and mycophenolate allow complete remission in most patients.- - - - - - - - - - ranking = 0.50078242443632keywords = oral ulcer, mouth (Clic here for more details about this article) |
4/30. An unusual case of pemphigus vulgaris presenting as bilateral foot ulcers.We describe an unusual presentation of pemphigus vulgaris, an autoimmune intraepidermal blistering skin disease associated with autoantibodies to the desmosome glycoprotein, desmoglein 3. A 60-year-old man presented with bilateral ulceration on the dorsum of the feet. These clinical features persisted for 4 months before more characteristic signs of pemphigus vulgaris, including mouth ulceration and skin erosions, developed. The atypical presentation led to a delay in diagnosis and initiation of the appropriate treatment. pemphigus vulgaris may have unusual manifestations, such as nail dystrophy, paronychia, or granulation tissue-like lesions, but this case of bilateral foot ulceration highlights a further, perhaps unique, clinical presentation of this autoimmune disease.- - - - - - - - - - ranking = 0.00078242443631746keywords = mouth (Clic here for more details about this article) |
5/30. Immunoablative high-dose cyclophosphamide without stem cell rescue in a patient with pemphigus vulgaris.The use of ablative intravenous cyclophosphamide (50 mg/kg per day for 4 days) without stem cell rescue has been described in patients with refractory autoimmune diseases such as paraneoplastic pemphigus, systemic lupus erythematosus, and aplastic anemia. We describe a 33-year-old patient with pemphigus vulgaris recalcitrant to multiple therapies. The patient presented with numerous oral ulcerations, erosions, and hyperpigmented crusted plaques on his face, trunk, and arms. Findings of a skin biopsy and direct immunofluorescence were consistent with pemphigus vulgaris. The circulating pemphigus vulgaris autoantibodies were present at a titer of 1:640. The patient received immunoablative therapy (50 mg/kg of cyclophosphamide for a total of 4 days) and tolerated the regimen well. Complications such as thrombocytopenia and pseudomonas septicemia were quickly treated. Four months after the 4-day therapy, his oral and skin lesions completely healed, and his pemphigus titers have decreased to zero. He is no longer receiving prednisone and no new lesions have developed. This provides further evidence that this regimen is relatively safe and provides a potential "cure" for refractory autoimmune diseases such as pemphigus vulgaris.- - - - - - - - - - ranking = 0.5keywords = oral ulcer (Clic here for more details about this article) |
6/30. A Case of pemphigus foliaceus which occurred after five years of remission from pemphigus vulgaris.A 77-year-old Japanese female developed pemphigus foliaceus (PF) after 5 years of remission from pemphigus vulgaris (PV). The patient had painful erosions in her mouth and flaccid blisters of the skin and was diagnosed as having PV, which responded well to corticosteroid treatment. She was then free from any lesion of PV for 5 years with a low dose of corticosteroid. Then she developed scaly erythematous lesions on the skin and was diagnosed as suffering from PF. enzyme-linked immunosorbent assay (ELISA) using recombinant desmoglein 1 (Dsg-1) and Dsg-3 revealed that she had anti-Dsg-3 IgG in the PV stage, no antibodies during remission and anti-Dsg-1 IgG in the PF stage. These findings indicate that the target antigen was shifted from Dsg-3 to Dsg-1 along with the phenotype after a 5-year interval in this patient.- - - - - - - - - - ranking = 0.00078242443631746keywords = mouth (Clic here for more details about this article) |
7/30. Squamous cell carcinoma of the oral cavity--chronic oral ulcerative disease as a possible etiologic factor.This report documents the association of carcinoma of the oral cavity with chronic oral ulcerative disease in two patients. This association has not previously been documented in the surgical literature. Both patients in this report had chronic oral ulcerative disease preceding their cancers; however, the common etiologic factors for oral cancer were not detected in either case. Oral lichen planus and pemphigus vulgaris should be considered as potentially premalignant lesions and should be treated accordingly.- - - - - - - - - - ranking = 3keywords = oral ulcer (Clic here for more details about this article) |
8/30. Oral pemphigus vulgaris occurring during pregnancy.There have been few reports describing the occurrence of pemphigus vulgaris (PV) during pregnancy. The patient described in this case report is interesting because the PV that developed during her pregnancy was confined to her mouth. It has been suggested that prompt treatment with systemic steroids prevents development of PV in cutaneous tissues. In this case, early control of the condition is believed to have eliminated the need for high dose steroids throughout the remainder of the pregnancy. In addition, this therapeutic approach could have contributed to the birth of a baby free of PV. Resolution of the presenting oral symptoms allowed the mother to resume a normal diet, allaying her anxiety about the possible effects of poor nutritional intake on foetal development. Aspects of clinical management considered in this report include the choice of immunosuppressive therapy and the multidisciplinary care involving both dental and obstetric specialists.- - - - - - - - - - ranking = 0.00078242443631746keywords = mouth (Clic here for more details about this article) |
9/30. The transition of pemphigus vulgaris into pemphigus foliaceus: a reflection of changing desmoglein 1 and 3 autoantibody levels in pemphigus vulgaris.The transition of pemphigus vulgaris (PV) into pemphigus foliaceus (PF) is rare and the immunological changes underlying this event are not well understood. We report a 44-year-old woman who presented with oral and cutaneous erosions typical of PV. Over a 9-year period, the clinical features evolved into those of PF. To examine whether quantitative changes in desmoglein (Dsg) antibodies were associated with this transition, Dsg1 and Dsg3 antibody levels were measured by enzyme-linked immunosorbent assay in 82 sequential serum samples collected over this period. At presentation, when the phenotype was PV with oral and cutaneous erosions, antibodies to both Dsg1 and Dsg3 were detected. The disappearance of oral involvement was associated with a decline in Dsg3 antibodies, which are now undetectable, while the development of more severe skin involvement was associated with rising Dsg1 antibody levels. These data strongly suggest that the change in clinical features is a reflection of qualitative and quantitative changes in antibody profile. It is not known whether the transition to PF is permanent or whether disease relapses in the future may be associated with the re-emergence of Dsg3 antibodies, oral ulceration and a PV phenotype.- - - - - - - - - - ranking = 0.5keywords = oral ulcer (Clic here for more details about this article) |
10/30. Paraneoplastic pemphigus in children and adolescents.BACKGROUND: Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease associated with specific B-cell lymphoproliferative neoplasms. There has been an increasing number of individual reports in the childhood and adolescent population. OBJECTIVES: To examine the clinical and immunopathological features of PNP occurring in children and adolescents. patients AND methods: We analysed the clinical and immunopathological findings of 14 patients under the age of 18 years with a confirmed diagnosis of PNP. Sera from all patients were analysed by indirect immunofluorescence (IF) and immunoprecipitation for plakin autoantibodies, immunoblotting for detection of plectin autoantibodies, and enzyme-linked immunosorbent assay (ELISA) for the detection of desmoglein (Dsg) 1 and Dsg3 autoantibodies. RESULTS: Severe oral mucositis was observed in all patients, and lichenoid cutaneous lesions in eight of 14 patients. The average age at presentation was 13 years. Striking findings included: pulmonary destruction leading to bronchiolitis obliterans in 10 patients, association with Castleman's disease in 12 patients, and a fatal outcome in 10 patients. The underlying neoplasm was occult in 10 patients. Histological findings include lichenoid and interface dermatitis with variable intraepithelial acantholysis. Deposition of IgG and C3 in the mouth and skin by direct IF was not found in some cases, but indirect IF detected IgG autoantibodies in all cases. immunoprecipitation revealed IgG autoantibodies against desmoplakin I, envoplakin and periplakin in all cases, and against desmoplakin II and the 170-kDa antigen in 13 and 10 patients, respectively. Dsg3 and Dsg1 autoantibodies were present in 10 and three patients, respectively, and plectin autoantibodies in 13 patients. CONCLUSIONS: PNP in children and adolescents is most often a presenting sign of occult Castleman's disease. It presents with severe oral mucositis and cutaneous lichenoid lesions. serum autoantibodies against plakin proteins were the most constant diagnostic markers. Pulmonary injury appears to account for the very high mortality rates observed.- - - - - - - - - - ranking = 0.00078242443631746keywords = mouth (Clic here for more details about this article) |
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