Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038362 (stomatitis)
 8,852 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A severe, extensive, erosive stomatitis developed in a 33-year-old Japanese man who had had intraperitoneal giant lymph node hyperplasia (Castleman's tumor) of the hyaline-vascular type. Histologically, there was a dense lymphoid cell infiltration in the dermis with invasion of the mucosal epithelium. It was hypothesized that an autoaggressive attack of lymphoid cells against the epithelium of the oral mucosa played a role in the pathogenesis of this erosive stomatitis and it was suggested that there might be a correlation between the occurrence of stomatitis and the presence of Castleman's tumor.
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PMID:Severe erosive stomatitis and giant lymph node hyperplasia of retroperitoneum (Castleman's tumor). 68 Feb 99

Castleman's disease is a rare, benign, lymphoproliferative disorder of unknown cause. The hyaline-vascular type is frequently associated with a localized mediastinal mass. The plasma-cell type is associated with constitutional symptoms, multicentric lymph node involvement, lymphoma development, and autoimmune disease-like laboratory abnormalities such as elevated erythrocyte sedimentation rate, anemia, and thrombocytopenia. We report a case of hyaline-vascular Castleman's disease associated with a cutaneous autoimmune disease, pemphigus vulgaris. We also reviewed the clinicopathologic features of four similar cases. Among these five reports of Castleman's disease, five patients had severe erosive stomatitis diagnosed as oral pemphigus, three had keratoconjunctivitis, and three had circulating pemphigus antibodies. All were young, ranging in age from 15 to 21 years, and four of the five were women. Two had hyaline-vascular Castleman's disease, whereas three had plasma-cell Castleman's disease. All five had surgical resection of the Castleman's disease mass. After surgery, remission of pemphigus vulgaris could be achieved with reduced dosages of steroids in all cases. In at least two cases steroid treatment could be completely discontinued. We postulate that an underlying immune dysfunction in Castleman's disease facilitates the expression of pemphigus.
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PMID:Castleman's disease associated with pemphigus vulgaris. 176 79

We present a case of paraneoplastic pemphigus associated with Castleman's disease. Our patient had stomatitis and vulvar erosions followed by a cutaneous polymorphous eruption. Investigations for neoplasia disclosed a Castleman's tumour suggesting the diagnosis of paraneoplastic pemphigus. The diagnosis was supported by immunoblotting using an extract of cow tongue although keratinocytes extracts did not identify relevant target antigens. One year after surgical excision of the tumour the patient remained unwell with persistent buccal erosions and lymphopenia. This case is unusual because of the length of its evolution before the discovery of the Castleman's tumour. It is only the second occasion that the association between paraneoplastic pemphigus and Castleman's tumour has been reported. Our case emphasizes the usefulness of immunoblotting on cow tongue in suspected cases of paraneoplastic pemphigus.
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PMID:Paraneoplastic pemphigus associated with Castleman's tumour. 1084

Among diagnostic progress over the last three years in internal medicine, Antisynthetase Syndrome is now more easily recognised with the diffusion of laboratory tests for research of antibodies against tRNA synthetases (Anti JO1, anti PL7, Anti PL12). In two third of cases, these antibodies are found despite absence of antinuclear antibodies. Hence, we have to search them specifically in patients with polyarthritis associated with myositis, cutaneous manifestations (Raynaud phenomenom and "mechanic'hands") and interstitial lung disease. Discovery of asymptomatic mutation in the L ferritin coding sequence help us to better understand the "unexplained" hyperferritinemia. Initially described by japonese gastroenterologists, auto immune pancreatitis in fact a part of a systemic sclerosing disease with a biochemical hallmark: in crease of a subclass of immunoglobulins G (IgG4). A new pediatric disease due to a deficiency of the interleukin1 receptor antagonist (multifocal aseptic osteitis, periostitis, stomatitis, disseminated pustulosis) help us to better understand unexplained auto inflammatory diseases. The therapeutic progress is primarily due to an explosion of biological therapies, particularly four of them very useful for internists (in an off label use) : Interleukin 1 inhibitors (anakinra, Canakinumab) to treat some auto inflammatory diseases (cryopirin associated periodic syndromes and deficency of interleukin 1 receptor antagonist), monoclonal antibody against interleukin 5 (mepolizumab) to treat some hypereosinophilic syndromes and Churg and Strauss angiitis, interleukin 6 inhibitiors to treat multifocal Castleman's disease and adult Still disease, a monoclonal antibody against vascular endothelial growth factor (Bevacizumab) to treat hereditary hemorrhagic telangiectasia.
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PMID:[What's new in internal medicine?]. 2011 57

Paraneoplastic pemphigus (PNP) is often a fatal autoimmune bullous disease characterized by severe stomatitis, polymorphous skin eruptions, and underlying neoplasms. We describe a patient with PNP associated with follicular dendritic cell sarcoma (FDCS), a rare neoplasm originating from follicular dendritic cells, which are non-lymphoid, non-phagocytic accessory cells of the lymphoid system and play an integral role in regulation of the germinal center reaction and present antigens to B-cells. The presence of rich vascularity around the tumor and few hyalanized vascular follicles found in histopathological examination gives the clue that the tumor might have developed from Castleman's disease (CD). As for the mechanisms by which CD induces PNP, it has been proposed that autoantibodies secreted from the Castleman's tumor play pivotal role. This hypothesis seems to be supported by the present case, in which CD may have triggered both the FDCS and the PNP.
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PMID:Follicular dendritic cell sarcoma with paraneoplatic pemphigus: Rare case and a brief review of literature. 2460 65