1/33. Scrape cytology of oral pemphigus. Report of a case with immunocytochemistry and light, scanning electron and transmission electron microscopy.BACKGROUND: pemphigus vulgaris is a disseminated disease of the skin and mucous membranes characterized by recurrent vesicular and bullous lesions due to the autoantigen belonging to the cadherin type of cell adhesion molecules. The presence of acantholysis associated with immunoglobulins in the intercellular spaces and on the cell membrane are diagnostic features. However, the appearance of smears from the oral cavity by scanning (SEM) and transmission electron microscopic (TEM) study as well as immunocytochemistry of cadherin does not appear to have been previously reported. CASE: A 67-year-old female developed erosion on her gingiva with severe pain. On oral examination, there were ulcerations on the palate, and the Nikolsky sign was positive. The characteristic cytologic findings from oral scrapes were high cellularity, a bloody background and a predominant cell population consisting of polygonal basal and parabasal cells with pronounced nucleoli. Also present were degenerative cell changes: e.g., cytoplasmic vacuoles and a homogeneous nuclear appearance. Immunocytochemical staining for IgG and cadherin gave a positive reaction in the intercellular spaces and on the cell membranes. The surface of cells in pemphigus vulgaris by SEM showed somewhat irregularly distributed microridges, and TEM revealed desmosomal attachments, degenerated tonofilaments with pronounced nucleoli and heterochromatin. As a result of cytodiagnosis, additional appropriate specimens were obtained at the time of the scraping for confirmatory immunocytochemistry for cadherin, SEM and TEM studies. CONCLUSION: The results demonstrate that a precise diagnosis of pemphigus vulgaris can be rendered on cellular material and cadherin immunocytochemistry obtained by scrape from the oral mucosa.- - - - - - - - - - ranking = 1keywords = oral cavity, cavity (Clic here for more details about this article) |
2/33. Paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis and bronchiolitis obliterans.BACKGROUND: Cases of paraneoplastic pemphigus (PNP) have been reported associated with various lymphoproliferative malignancies and benign Castleman tumors, with the most severe course and fatal outcome seen in patients with bronchiolitis obliterans. OBJECTIVE: The aim was to establish immunologic associations by coexistence of Castleman tumor, myasthenia gravis, and bronchiolitis obliterans and to evaluate the treatment modalities. methods: Clinical studies included computed tomography of the mediastinum, computed tomography and magnetic resonance imaging of the abdominal cavity, and quantitative electromyography. Direct and indirect immunofluorescence on various substrates, immunoblot analysis, immunoprecipitation, and specific enzyme-linked immunosorbent assay using recombinant desmogleins (Dsg) were performed as immunologic assays. RESULTS: Direct and indirect immunofluorescence including rat bladder showed intercellular antibodies. immunoblotting disclosed antibodies to envoplakin (210 kd protein) and periplakin (190 kd protein); in addition, immunoprecipitation detected antibodies to desmoplakin I (250 kd protein). Antibodies to Dsg3 (pemphigus vulgaris antigen) were detected by specific enzyme-linked immunosorbent assay. myasthenia gravis was controlled by drugs; however, mucocutaneous changes were not fully responsive to corticosteroids and cyclophosphamide pulses, cyclosporine, and intravenous immunoglobulins. The surgical removal of Castleman tumor did not change the course of the disease. The fatal outcome was the result of bronchiolitis obliterans that occurred after the surgery and was only transitionally controlled by plasmapheresis. CONCLUSION: This is the first case of paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis, and bronchiolitis obliterans. Despite a benign character of the tumor the patient died, as do all patients with bronchiolitis obliterans. Massive plasmapheresis has only a transient effect. We confirmed the presence of antibodies to Dsg 3, in addition to the set of specific paraneoplastic pemphigus antibodies against various proteins of plakin family.- - - - - - - - - - ranking = 0.021633112781499keywords = cavity (Clic here for more details about this article) |
3/33. Anaesthetic management of a patient with pemphigus vulgaris for emergency laparotomy.A 45-year-old man with a long-standing history of duodenal ulcer presented with symptoms and signs of perforation peritonitis. He also had lesions of pemphigus vulgaris throughout the body, involving both skin and mucous membranes. Care was taken to avoid pressure and friction during placement of monitoring devices, intravenous and arterial lines. Since the patient had to undergo exploratory laparotomy, intubation was performed in an atraumatic manner after rapid sequence induction. However, there was minor bleeding from the mucous lesions of the oral cavity, which was controlled by a saline adrenaline throat pack. The patient was extubated at the end of the surgery and steroids were continued in the peri-operative period.- - - - - - - - - - ranking = 0.021633112781499keywords = cavity (Clic here for more details about this article) |
4/33. Oral acantholytic itching disease responding to dapsone. dermatitis herpetiformis, pemphigus, or a new disease?A patient had a blistering and severely itching disease confined solely to the oral cavity. The histopathologic findings had the features of both pemphigus and dermatitis herpetiformis. There were no gastrointestinal symptoms, no IgA could be found in uninvolved skin, and no intercellular or basement membrane antibodies were present in the serum or epidermis. The disease responded favorable to dapsone (diaminodiphenylsulfone), which could be discontinued after 2 1/2 years. The literature is briefly reviewed, and there is a discussion of whether the patient was suffering from pemphigus or dermatitis herpetiformis. The conclusion is drawn that the diagnosis cannot be established with certainty and that we are perhaps dealing with a new disease. It is stressed that in such aberrant cases a therapeutic trial with dapsone should be made.- - - - - - - - - - ranking = 1keywords = oral cavity, cavity (Clic here for more details about this article) |
5/33. 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 = 0.025961537271546keywords = mouth (Clic here for more details about this article) |
6/33. 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 = 0.025961537271546keywords = mouth (Clic here for more details about this article) |
7/33. Paraneoplastic pemphigus caused by an epithelioid leiomyosarcoma and associated with fatal respiratory failure.A patient is described who initially presented with pemphigus vulgaris, limited to the oral cavity, and weight loss. Although the various laboratory studies pointed to the diagnosis of paraneoplastic pemphigus (PNP), the underlying neoplasm was not detected until 6 months later, when the patient developed shortness of breath and routine physical examination on admission revealed an abdominal mass, which eventually was proven to be an epithelioid leiomyosarcoma. In spite of radical excision of the tumour and intensive treatment of the dyspnoea, the patient died of respiratory failure 19 months after the PNP had been diagnosed. early diagnosis of PNP is stressed to possibly prevent fatal pulmonary involvement.- - - - - - - - - - ranking = 1keywords = oral cavity, cavity (Clic here for more details about this article) |
8/33. Paraneoplastic pemphigus in association with a retroperitoneal Castleman's disease presenting with a lichen planus pemphigoides-like eruption. A case report and review of literature.A 50-year-old man presented with severe mucosal erosions of the lips, oral cavity and perianal area, a lichen planus-like eruption on the trunk and extremities and scaly plaques of the palms and soles. The clinical impression was of Stevens--Johnson syndrome, or paraneoplastic pemphigus (PNP). Histopathology revealed vacuolar interface and lichenoid dermatitis with dyskeratosis and suprabasal acantholytic vesiculation. Direct immunofluorescence showed deposition of IgG in the intercellular space and linear deposition of C3 along the basal membrane zone. Indirect immunofluorescence revealed circulating IgG with intercellular staining of the epithelium of rat urinary bladder. Western blotting demonstrated bands of 250- and 230-kDa antigens. The clinical, histological and immunological features were consistent with the lichen planus pemphigoides variant of PNP. A retroperitoneal hyaline-vascular Castleman's disease was detected and excised. The skin lesions worsened initially after tumour resection but improved gradually, leaving extensive melanosis after cyclosporin and mycophenolate mofetil treatment.- - - - - - - - - - ranking = 1keywords = oral cavity, cavity (Clic here for more details about this article) |
9/33. 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 = 0.025961537271546keywords = mouth (Clic here for more details about this article) |
10/33. 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 = 5keywords = oral cavity, cavity (Clic here for more details about this article) |
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