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)

We herein firstly reported that a patient with thymoma-associated pemphigus (TAP) underwent a robotic-assisted trans-subxiphoid thoracoscopic extended thymectomy and then achieved stable resolution. The patient, a 47-year-old male, was first admitted to our hospital owing to stomatitis and bullae of the trunk after four months' prednisone treatment. On admission, chest computed tomography (CT) revealed an anterior-mediastinal mass and it was initially diagnosed as a thymoma. He was positive for anti-BP (bullous pemphigoid)-180 antibody and anti-desmoglein 3 antibody. Then, a robotic-assisted thymectomy was performed, following which, the anti-BP-180 and anti-desmoglein 3 antibody titres have declined. The patient's mucocutaneous lesions improved, and the steroid dose was gradually decreased from 60 to 40 mg/day. According to previous reports, and the experience of the presented case, we therefore believe that early extended thymectomy is an effective therapeutic intervention for TAP.
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PMID:Robotic trans-subxiphoid extended thymectomy in a patient with thymoma-associated pemphigus. 2874 Jun 98

Paraneoplastic autoimmune multiorgan syndrome (PAMS) is characterized by a heterogenous group of signs and symptoms including severe desquamative stomatitis, a polymorphous cutaneous eruption, humoral immunity against plakin proteins, contribution of cell-mediated autoimmunity and commonly a progressive respiratory failure. Autoantibodies in PAMS target a wide array of antigens including plakins, cadherins, alpha-2-macroglobulin like 1 (A2ML1), BP180, plakophilin-3, and several neuromuscular antigens. Originally described as paraneoplastic pemphigus in 1990 due to some of its clinical and immunologic similarities to classic pemphigus (pemphigus vulgaris and pemphigus foliaceus), PAMS is a multiorganopathy with several distinct features from these classic forms of pemphigus. Epidemiologically, PAMS is associated with underlying neoplasia and has a differing HLA-II allele predisposition compared to classic forms of pemphigus. Clinically, lesion morphology is polymorphous, and lesion distribution fundamentally differs from that seen in classic pemphigus. PAMS has a significantly higher mortality rate and a poorer response to treatments typically effective in pemphigus. Histologically, PAMS is characterized by the presence of interface dermatitis, vacuolar changes, and dyskeratotic keratinocytes which are not seen in classic pemphigus. PAMS demonstrates not only intercellular IgG as seen in classic pemphigus, but the presence of linear basement membrane zone deposition. Antibodies against desmoglein 3 (Dsg3) map to a broader array of epitopes than in pemphigus vulgaris and there is a higher prevalence of complement binding anti-Dsg3 IgG autoantibodies in PAMS. Autoantibodies can in rare cases be absent in the more cell-mediated form of PAMS. Considering these numerable differences, we review the entity of PAMS, and provide similarities and differences to classic forms of pemphigus.
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PMID:Paraneoplastic autoimmune multiorgan syndrome (PAMS): Beyond the single phenotype of paraneoplastic pemphigus. 3010 46