RESUMEN
Woringer-Kolopp disease, also known as pagetoid reticulosis, is an exceedingly rare variant of mycosis fungoides. Accurate diagnosis and effective treatment is essential to prevent progression to debilitating disease. We identified 7 patients with Woringer-Kolopp disease treated at our institution. We review the major clinical and pathologic characteristics of this disease, focusing on treatment strategies and patient outcomes. All of our patients were successfully treated with skin-directed therapies including topical steroids, topical nitrogen mustard, psoralen plus ultraviolet A, narrow-band ultraviolet B, and radiation therapy. Our observations confirm that Woringer-Kolopp disease carries an excellent prognosis, and support that the most effective and appropriate treatment for recalcitrant or severe Woringer-Kolopp disease is localized radiation therapy.
Asunto(s)
Micosis Fungoide/diagnóstico , Micosis Fungoide/patología , Recurrencia Local de Neoplasia/tratamiento farmacológico , Neoplasias Cutáneas/diagnóstico , Neoplasias Cutáneas/patología , Adolescente , Adulto , Anciano , Biopsia , Niño , Diagnóstico Diferencial , Femenino , Dermatosis de la Mano/diagnóstico , Humanos , Lactante , Masculino , Mecloretamina/administración & dosificación , Micosis Fungoide/tratamiento farmacológico , Micosis Fungoide/radioterapia , Recurrencia Local de Neoplasia/patología , Terapia PUVA , Fotones/uso terapéutico , Dosis de Radiación , Terapia Recuperativa , Piel/patología , Neoplasias Cutáneas/tratamiento farmacológico , Neoplasias Cutáneas/radioterapia , Resultado del TratamientoRESUMEN
Cutaneous plasmacytosis is a rare disorder that typically affects middle-aged to older individuals of Asian, particularly Japanese, descent. Clinically, it is characterized by multiple asymptomatic red-brown plaques and nodules on the trunk. Lymphadenopathy and hypergammaglobulinemia may be present. Histologically, the lesions show a moderately dense superficial and deep perivascular infiltrate composed predominantly of mature plasma cells without atypia or light chain restriction. We report our experience with five additional cases, including results of immunohistochemical studies for human herpes virus 8.