RESUMO
Sézary syndrome is a rare aggressive leukemic variant of primary cutaneous T-cell lymphoma, typically presenting with erythroderma, lymphadenopathy, and an atypical clonal T-cell population. Though it often involves the spleen and liver, we report a case of Sézary syndrome with renal involvement that was treated successfully. Visceral involvement confers a poor prognosis requiring systemic treatment. The patient we describe was a 66-year-old man who was referred from Dermatology services for deteriorating kidney function. Polymerase chain reaction of genomic DNA from skin and kidney biopsies confirmed a clonal T-cell population matching a population isolated in peripheral blood. The patient was treated initially with alemtuzumab, which led to a significant improvement in kidney function, and he has subsequently received a successful allogeneic stem cell transplant. This case represents a rare cause of decreased kidney function and highlights the role of biopsy in patients with suspected Sézary syndrome.
Assuntos
Alemtuzumab/administração & dosagem , Transplante de Células-Tronco Hematopoéticas/métodos , Neoplasias Renais/secundário , Síndrome de Sézary/patologia , Neoplasias Cutâneas/patologia , Idoso , Biópsia por Agulha , Terapia Combinada , Seguimentos , Humanos , Imuno-Histoquímica , Testes de Função Renal , Neoplasias Renais/terapia , Masculino , Síndrome de Sézary/terapia , Neoplasias Cutâneas/terapia , Transplante Homólogo , Resultado do TratamentoRESUMO
The use of medicinal plants is as old as the hills (and valleys and plains) where they grow. In this brief review, we have set out to emphasize the need for rigor in the assessment of any medicines-whatever their source-as we seek to translate apparently important observations on the efficacy of plant concoctions into real-time clinical practice for the benefit of our fellow men and women. We shall use examples of recently studied compounds that have touched the world of dermatology to illustrate some of the pitfalls and also to draw attention to some of the successes in moving from "folklore to pharmacy."
Assuntos
Fitoterapia , Dermatopatias/tratamento farmacológico , Ciclosporina , Diterpenos , Descoberta de Drogas , Medicamentos de Ervas Chinesas , Humanos , Ácidos Linoleicos , Oenothera biennis , Papaverina , Óleos de Plantas , Ácido gama-LinolênicoRESUMO
Erythrokeratodermia variabilis (EKV) is a rare genodermatosis with a unique phenotype. Treatment with oral synthetic retinoids is well documented in adults, but not in children. We report a 9-year-old girl with EKV who cleared rapidly following treatment with oral acitretin.