Paraneoplastic Pemphigus. A Life-Threatening Autoimmune Blistering Disease / Pénfigo paraneoplásico. Una enfermedad ampollosa autoinmune grave

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

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

Paraneoplastic 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. 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.

Efficacy and safety of azathioprine and dapsone as an adjuvant in the treatment of bullous pemphigoid

Background: Bullous pemphigoid is a chronic, blistering and autoimmune disease, common in old age. The treatment usually includes systemic steroids, however, these cause high morbidity rates, and then different products that function as adjuvants have been tried. At present, there are no studies to determine which adjuvant offers a better efficacy and safety profile. Methods: We performed a retrospective study which included the records of patients with bullous pemphigoid, treated either with azathioprine or dapsone. We evaluated the time to achieve complete remission, the time to inhibit disease progression, and the control of pruritus. Results: Fifteen records of patients were selected, eight (53%) treated with azathioprine and seven (47%) with dapsone. Complete remission was achieved at week six in both groups. We found no difference in the inhibition of disease progression (p = 0.083). Pruritus was controlled at four weeks of treatment in both treatments. Conclusions: Both products are effective as adjuvant in the treatment of bullous pemphigoid, with an acceptable safety profile(AU)

Efficacy and safety of azathioprine and dapsone as an adjuvant in the treatment of bullous pemphigoid.

BACKGROUND: Bullous pemphigoid is a chronic, blistering and autoimmune disease, common in old age. The treatment usually includes systemic steroids, however, these cause high morbidity rates, and then different products that function as adjuvants have been tried. At present, there are no studies to determine which adjuvant offers a better efficacy and safety profile. METHODS: We performed a retrospective study which included the records of patients with bullous pemphigoid, treated either with azathioprine or dapsone. We evaluated the time to achieve complete remission, the time to inhibit disease progression, and the control of pruritus. RESULTS: Fifteen records of patients were selected, eight (53%) treated with azathioprine and seven (47%) with dapsone. Complete remission was achieved at week six in both groups. We found no difference in the inhibition of disease progression (p=0.083). Pruritus was controlled at four weeks of treatment in both treatments. CONCLUSIONS: Both products are effective as adjuvant in the treatment of bullous pemphigoid, with an acceptable safety profile.

Treatment of pemphigus vulgaris. An overview in Mexico.

Pemphigus is an autoimmune skin disease that can present in a variety of forms and can prove challenging to manage and treat. An overview of the condition in Mexico is presented. Emphasis is placed on management of the condition, with description of the most commonly used treatments (glucocorticoids, azathioprine), the second line therapies (cyclosporine and mycophenolate mofetil), and additional alternative treatments (cyclophosphamide and dapsone).

Treatment of pemphigus vulgaris. An overview in Mexico / Tratamiento del pénfigo vulgar. Estado actual en méxico

Pemphigus is an autoimmune skin disease that can present in a variety of forms and can prove challenging to manage and treat. An overview of the condition in Mexico is presented. Emphasis is placed on management of the condition, with description of the most commonly used treatments (glucocorticoids, azathioprine), the second line therapies (cyclosporine and mycophenolate mofetil), and additional alternative treatments (cyclophosphamide and dapsone)

El pénfigo es una enfermedad autoinmune de la piel que se presenta en una variedad de formas y puede ser un reto para manejar y tratar. Se presenta un panorama de la enfermedad. Se hace énfasis en lo terapéutico. Están incluidas las opciones de tratamiento más comúnmente usadas (glucocorticoides y azatioprina), algunas alternativas (ciclosporina y mofetil micofenolato) y alternativas adicionales (ciclofosfamida y dapsona)

Disfunción eréctil durante el tratamiento con isotretinoína / Erectile dysfunction during isotretinoin therapy

La isotretinoína es un medicamento muy efectivo para el manejo del acné severo resistente al tratamiento convencional. Sin embargo, su uso se encuentra asociado con numerosos efectos secundarios, algunos de ellos de índole psiquiátrica. Durante la realización de un estudio prospectivo para evaluarla eficacia y seguridad de la isotretinoína en pacientes adultos con acné severo, seis pacientes hombres, refirieron dificultades para mantener una adecuada erección del pene asociado a datos clínicos de depresión. En nuestro conocimiento ésta es la primera vez que se reporta este efecto colateral, así que los resultados sugieren que se deben realizar más estudios de su uso, investigando específicamente este efecto secundario (AU)

Isotretinoin is a very effective drug for the treatment of severe recalcitrant acne. However, it’s use is associated with many side effects, sometimes is associated with the emergence of psychiatric symptoms. During of a prospective study to evaluate the efficacy and safety of isotretinoin in acne, six male patients, refer difficulties in maintaining adequate penile erection in association with clinical symptoms of depression. To our knowledge this is the first time that this side effect is reported, so the results suggest that further studies of it’s use in similar patients are warranted (AU)

[Erectile dysfunction during isotretinoin therapy]. / Disfunción eréctil durante el tratamiento con isotretinoína.

Isotretinoin is a very effective drug for the treatment of severe recalcitrant acne. However, it's use is associated with many side effects, sometimes is associated with the emergence of psychiatric symptoms. During of a prospective study to evaluate the efficacy and safety of isotretinoin in acne, six male patients, refer difficulties in maintaining adequate penile erection in association with clinical symptoms of depression. To our knowledge this is the first time that this side effect is reported, so the results suggest that further studies of it's use in similar patients are warranted.