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  • 1
    Electronic Resource
    Electronic Resource
    (3) Calcium enhances the sensitivity of immunofluorescence for pemphigus antibodies (1988)
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 119 (1988), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2133.1988.tb05377.x
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  • 2
    Electronic Resource
    Electronic Resource
    Paraneoplastic pemphigus in children and adolescents (2002)
    Oxford, UK : Blackwell Science Ltd
    British journal of dermatology 147 (2002), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary 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.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2133.2002.04992.x
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  • 3
    Electronic Resource
    Electronic Resource
    Anti-epiligrin (laminin 5) cicatricial pemphigoid (1997)
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 137 (1997), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Cicatricial pemphigoid is an autoimmune disease predominantly of elderly patients. Recently, a subtype of cicatricial pemphigoid with antibodies to epiligrin (laminin 5) was described. We report a patient in whom anti-epiligrin cicatricial pemphigoid was identified. He presented with cutaneous, oral and ocular involvement. He has shown initial improvement on corticosteroids and cyclophosphamide.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2133.1997.20002081.x
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  • 4
    Electronic Resource
    Electronic Resource
    Treatment with alpha interferon associated with the development of paraneoplastic pemphigus (1995)
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 132 (1995), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: The use of interferon (INF) is usually considered safe, the major side-effect being a flu-like syndrome. However, with its ability to alter immune responsiveness, INF has been associated with the induction of autoimmune diseases.We report a patient with Waldenström's macroglobulinaemia who developed a generalized blistering eruption after treatment with systemic INF-α 2A (INF) for multiple skin cancers. The patient's skin showed histological features, immunofluorescence findings, and immunoprecipitation diagnostic of paraneoplastic pemphigus. Despite aggressive treatment the patient died.In our patient, the use of INF was temporally related to the development of paraneoplastic pemphigus. Although interferon has been shown to induce other autoimmune diseases, to our knowledge this is only the second report of a patient treated with an interferon who subsequently developed a fatal autoimmune blistering disorder. Paraneoplastic pemphigus is a recently described autoimmune bullous disorder with a poor prognosis. The mechanism by which INF triggered the paraneoplastic pemphigus is not known.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2133.1995.tb08688.x
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  • 5
    Electronic Resource
    Electronic Resource
    Elevated thymosin alpha I levels in Brazilian pemphigus foliaceus (1986)
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 115 (1986), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Levels of thymosin alpha I in the sera of 37 patients with Brazilian pemphigus foliaceus (BPF) were measured using a competitive binding radioimmunoassay. The values were compared with 19 patients with other forms of pemphigus, 13 relatives of patients with BPF, 18 patients with other dermatological diseases, and 265 normal controls. We found that 27 (73%) of the patients with BPF had thymosin alpha I serum levels that were at least two standard deviations above the mean for normal individuals. The mean value for patients with BPF was significantly greater than any other groups studied. The thymosin elevation is similar to alterations seen in certain viral diseases and suggests that BPF is aetiopathogenically distinct from the other forms of pemphigus.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2133.1986.tb05710.x
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  • 6
    Electronic Resource
    Electronic Resource
    Paraneoplastic pemphigus: the role of tumours and drugs (2001)
    Oxford, UK : Blackwell Science Ltd
    British journal of dermatology 144 (2001), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2133.2001.04272.x
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  • 7
    Electronic Resource
    Electronic Resource
    Infliximab for peristomal pyoderma gangrenosum (2003)
    Oxford, UK : Blackwell Science Ltd
    British journal of dermatology 148 (2003), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary Peristomal pyoderma gangrenosum (PPG) is a variant of pyoderma gangrenosum (PG) that is more refractory to treatment. It is a cause of severe morbidity and poses a therapeutic challenge for the clinician. Infliximab (Remicade®; Centocor, Malvern, PA, USA) is a chimeric monoclonal antibody directed against tumour necrosis factor-α that has been proven to be effective in the treatment of inflammatory bowel disease (IBD) and rheumatoid arthritis. Currently, very few reports exist documenting its use in the treatment of PG and none in the treatment of PPG. We describe our experience of treating three patients with IBD-associated PPG with infliximab. All patients tolerated the drug without significant side-effects. Two patients with PPG recovered completely following the administration of infliximab, and one patient had a partial response to the drug. We conclude that infliximab appears to be a safe and effective therapeutic alternative in patients with PPG.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2133.2003.05294.x
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  • 8
    Electronic Resource
    Electronic Resource
    Paraneoplastic pemphigus without antidesmoglein 3 or antidesmoglein 1 autoantibodies (2001)
    Oxford, UK : Blackwell Science Ltd
    British journal of dermatology 144 (2001), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous blistering disease characterized by IgG autoantibodies that bind to various epithelia and immunoprecipitate a complex of 250, 230, 210, 190 and 170 kDa proteins. A recent study has suggested that PNP patients have antidesmoglein (Dsg) 3 autoantibody and that the antibody plays a pathogenic role in PNP. We report a 72-year-old woman with PNP associated with thymoma and adenocarcinoma of the lung. Diagnosis of PNP was made by the characteristic clinical, histological and immunopathological findings, as well as immunoprecipitation of characteristic 230, 210 and 190 kDa proteins. Using enzyme-linked immunosorbent assay with baculovirus-expressed recombinant proteins, the patient’s serum was negative against both Dsg 3 and Dsg 1. This finding is unusual, and it suggests that the target antigen, which is involved in acantholysis, may be other than Dsg 3 in this case.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2133.2001.04095.x
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  • 9
    Electronic Resource
    Electronic Resource
    Paraneoplastic pemphigus in association with Castleman's disease (2003)
    Oxford, UK : Blackwell Science Ltd
    British journal of dermatology 149 (2003), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Background  Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease associated with lymphoproliferative neoplasms, and frequently with a very rare tumour, Castleman's disease.Objectives  To analyse the clinical history, immunopathological and histopathological findings in 28 patients with a confirmed diagnosis of PNP and Castleman's disease.Methods  Sera from all patients were assayed by indirect immunofluorescence (IF) and immunoprecipitation (IP) for plakin autoantibodies, immunoblotting for detection of plectin autoantibodies, and enzyme-linked immunosorbent assay for detection of desmoglein (Dsg)1 and Dsg3 autoantibodies.Results  Severe oral mucositis was observed in all patients, and lichenoid cutaneous lesions were seen in 19 of 28. Twenty cases of Castleman's disease were of the hyaline vascular type, four were of plasmacytoid type and four were of mixed type. Striking findings included pulmonary destruction leading to bronchiolitis obliterans in 26 patients and fatal outcome due to respiratory failure in 22 patients with pulmonary involvement. Histological findings included lichenoid and interface dermatitis with variable intraepithelial acantholysis. Direct IF showed deposition of IgG and C3 in the mouth and skin in 24 of 28 patients. However, indirect IF detected serum IgG autoantibodies in all patients. IP revealed IgG autoantibodies against desmoplakin I, envoplakin and periplakin in all cases, and against desmoplakin II and the 170-kDa antigen in 19 patients. Dsg3 and Dsg1 autoantibodies were present in 22 and 11 patients, respectively, and plectin autoantibodies in 23 patients.Conclusions  PNP in association with Castleman's disease presents with severe oral mucositis and cutaneous lichenoid lesions. Serum autoantibodies against plakin proteins are the most diagnostic markers. Pulmonary injury with respiratory failure is the cause of death in most cases.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2133.2003.05659.x
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