1/5. Paraneoplastic pemphigus in a patient with a thymoma.A 76-year-old woman, with a history of thymoma, presented with a painful extensive stomatitis, painful paronychia, lichenoid papules on the hands and superficial erosions on the neck and the trunk. Histological examination showed lichenoid changes, acantholytic blister formation and apoptotic keratinocytes. Direct immunofluorescence was positive for IgG both in the epidermal intercellular spaces and along the basement membrane zone. Indirect immunofluorescence was similarly positive in a pemphigus vulgaris pattern. There was only a partial response to intravenous corticoids. These findings allowed the diagnosis of paraneoplastic pemphigus. The diagnostic characteristics, histopathology and the differential diagnosis of this disease are discussed.- - - - - - - - - - ranking = 1keywords = vulgaris (Clic here for more details about this article) |
2/5. Paraneoplastic pemphigus. An autoimmune mucocutaneous disease associated with neoplasia.BACKGROUND and methods. We describe five patients with underlying neoplasms in whom painful mucosal ulcerations and polymorphous skin lesions developed, usually with progression to blistering eruptions on the trunk and extremities. Histologic examination showed vacuolization of epidermal basal cells, keratinocyte necrosis, and acantholysis. Immunofluorescence testing revealed atypical pemphigus-like autoantibodies in perilesional epithelium and serum from all five patients. We studied the antigenic specificities of the autoantibodies by indirect immunofluorescence and immunoprecipitation, using extracts of 14C-labeled human keratinocytes. IgG purified from the serum of one patient was passively transferred to four neonatal mice to test for pathogenicity. RESULTS. Immunofluorescence testing showed that the autoantibodies bound to the surface of tissues containing desmosomes, including complex and simple epithelia, and myocardium. An identical and unique complex of four polypeptides with molecular weights of 250, 230, 210, and 190 was immunoprecipitated by all serum samples. The 250-kd polypeptide comigrated with desmoplakin I (a protein found in the desmosomes of all epithelia), and the 230-kd antigen comigrated with the antigen of bullous pemphigoid. Cutaneous blisters, a positive Nikolsky's sign, and epidermal and esophageal acantholysis developed in all mice into which the autoantibody was injected. Electron microscopy showed epidermal acantholysis similar to lesions of experimentally induced pemphigus vulgaris. CONCLUSION. These five patients with cancer had a novel acantholytic mucocutaneous disease characterized by autoantibodies that were pathogenic after passive transfer. The autoantibodies from these patients reacted with an antigen complex composed of desmoplakin I and the 230-kd antigen of bullous pemphigoid and two as yet unidentified epithelial antigens. We suggest the term "paraneoplastic pemphigus" for this disease.- - - - - - - - - - ranking = 1keywords = vulgaris (Clic here for more details about this article) |
3/5. recurrence of thymoma with appearance of myasthenia gravis 18 years after surgery: a case report.A 59-year-old woman was admitted to the hospital because of recurrence of thymoma with appearance of myasthenia gravis 18 years after excision of an invasive thymoma. recurrence was observed in the anterior mediastinum, right pleura, pericardium and left supraclavicular lymph node. Hashimoto's thyroiditis, vitiligo vulgaris and leukopenia were also seen. After creation of a pericardial window for pericardial effusion, she received vincristine, then carbazilquinone intravenously as single chemotherapeutic agents based on the chemosensitivity by clonogenic assay of the supraclavicular tumor, but response was not achieved. At one and a half years after radiotherapy to the mediastinum, she is now doing well without symptoms and is receiving a fairly low dose of anticholinesterase medication.- - - - - - - - - - ranking = 1keywords = vulgaris (Clic here for more details about this article) |
4/5. pemphigus vulgaris associated with spindle cell thymoma.This paper describes the occurrence of fatal pemphigus vulgaris in an 89 year old female, in whom a benign spindle cell thymoma was found at autopsy. The histological appearances of the skin biopsies, thymic tumour and immunofluorescence findings are described. The association between pemphigus and neoplasia is discussed, and the difference between classical pemphigus vulgaris and paraneoplastic pemphigus are evaluated.- - - - - - - - - - ranking = 6keywords = vulgaris (Clic here for more details about this article) |
5/5. Immunologic studies of a case of myasthenia gravis associated with pemphigus vulgaris after thymomectomy.A case of myasthenia gravis was associated with thymoma and pemphigus vulgaris. The bullous lesions developed after partial thymomectomy, cobalt (60Co) irradiation, and 3 days' extensive sunbathing, although a retrospective study of the patient's sera by quantitative indirect immunofluorescence method indicated that antiepithelial antibody already was positive before the clinical appearance of pemphigus vulgaris. Serial observation of the patient's clinical course and titrations of antiepithelial, antimuscle, and antithymus antibodies suggested a reverse relationship between the severity of myasthenia gravis and titers of antimuscle and anitithymus antibodies, and a parallel relationship between pemphigus vulgaris and antiepithelial antibody. review of the literature suggests a close relationship between pemphigus vulgaris and myasthenia gravis and thymoma, particularly thymoma injured by medical procedures.- - - - - - - - - - ranking = 8keywords = vulgaris (Clic here for more details about this article) |