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Vol. 108. Num. 10.December 2017
Pages 889-970
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Vol. 108. Num. 10.December 2017
Pages 889-970
Review
DOI: 10.1016/j.adengl.2017.04.011
Paraneoplastic Pemphigus. A Life-Threatening Autoimmune Blistering Disease
Pénfigo paraneoplásico. Una enfermedad ampollosa autoinmune grave
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A. Tirado-Sánchez
Corresponding author
atsdermahgm@gmail.com

Corresponding author.
, A. Bonifaz
Servicio de Dermatología, Hospital General de México, Mexico
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Table 1. Curth's criteria for the diagnosis of cutaneous paraneoplastic syndrome.
Table 2. Characteristics of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome.
Table 3. Differential diagnosis of PNP.
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Abstract

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. The earliest and most consistent finding is a painful, severe, chronic and often recalcitrant stomatitis. Treatment of PNP is difficult. Immunosuppressive agents are required to decrease blistering, and treating the underlying tumor may control autoantibody production. In this review, we included essential diagnostic aspects of PNP and the most useful treatment options in the dermatologist practice.

Keywords:
Paraneoplastic pemphigus
Rituximab
Antibody production
Lymphoproliferative origin
Prognosis
Pemphigus
Resumen

El pénfigo paraneoplásico (PNP), una variedad de pénfigo, es una enfermedad ampollosa autoinmune que puede afectar a múltiples órganos distintos de la piel. Es una enfermedad grave asociada con una malignidad subyacente, comúnmente de origen linfoproliferativo. Las lesiones clínicas pueden parecerse al pénfigo, penfigoide, eritema multiforme, enfermedad de injerto contra huésped o liquen plano. El hallazgo más temprano y más consistente es una estomatitis dolorosa, grave, crónica y, a menudo, recalcitrante. El tratamiento del PNP es difícil. Se requieren agentes inmunosupresores para disminuir la formación de ampollas y el tratamiento del tumor subyacente puede controlar la producción de autoanticuerpos. En esta revisión se incluyeron los aspectos diagnósticos más esenciales del PNP y las opciones de tratamiento más útiles en la práctica dermatológica.

Palabras clave:
Pénfigo paraneoplásico
Rituximab
Producción de anticuerpos
Origen linfoproliferativo
Pronóstico
Pénfigo
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