Paraneoplastic Pemphigus: Paraneoplastic Autoimmune Disease of the Skin and Mucosa
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چکیده
منابع مشابه
Paraneoplastic Pemphigus: A Paraneoplastic Autoimmune Multiorgan Syndrome or Autoimmune Multiorganopathy?
Paraneoplastic pemphigus (PNP), a clinically and immunopathologically distinct mucocutaneous blistering dermatosis, is a severe form of autoimmune multiorgan syndrome generally associated with poor therapeutic outcome and high mortality. This IgG-mediated disease is initiated by an obvious or occult lymphoproliferative disorder in most cases. Clinically severe mucositis, and polymorphic blister...
متن کاملParaneoplastic Pemphigus. A Life-Threatening Autoimmune Blistering Disease.
Paraneoplastic pemphigus (PNP), a subset of pemphigus, is a unique autoimmune blistering condition that can affect multiple organs other than the skin. It is a life-threatening disease associated with an underlying malignancy, most commonly of lymphoproliferative origin. The clinical picture may resemble pemphigus, pemphigoid, erythema multiforme, graft-versus-host disease, or lichen planus. Th...
متن کامل[Paraneoplastic pemphigus].
UNLABELLED A RARE DISEASE: Paraneoplastic pemphigus is an rare autoimmune bullous skin disease recently recognized. About 50 cases have been reported since its first description in 1990. CLINICAL MANIFESTATIONS Clinical signs are polymorphous resembling the cutaneomucosal manifestations of pemphigus vulgar (skin and mucosa erosions, fragile interdermal bullae), pemphigoid (urticaria, distende...
متن کاملParaneoplastic autoimmune multiorgan syndrome (paraneoplastic pemphigus) in a child: case report and review of the literature.
Paraneoplastic autoimmune multiorgan syndrome, also known as paraneoplastic pemphigus, has been observed only rarely among children. We describe a 10-year-old boy with typical clinical and histologic findings of paraneoplastic pemphigus associated with Castleman's disease. His disease was refractory to resection of the tumor and aggressive combination immunosuppressive therapies. The patient di...
متن کاملParaneoplastic Pemphigus: Recent Advances
These confirmatory reports have verified all the essential features of the disease that was first described. Mucosal ulceration in the form of intractable stomatitis is the most constant clinical feature of the disease. In no case to-date has this been absent. Cutaneous manifestations including blisters, lichenoid eruption or erythema multiforme like lesions are highly variable. These can chang...
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ژورنال
عنوان ژورنال: Frontiers in Immunology
سال: 2019
ISSN: 1664-3224
DOI: 10.3389/fimmu.2019.01259