RESUMEN
BACKGROUND: Intravascular lymphoma is a rare form of non-Hodgkin's lymphoma with varied but highly evocative clinical presentations, particularly in terms of dermatology. Histological examination of the skin may be sufficient to allow diagnosis. PATIENTS AND METHODS: Herein, we report the case of a 75-year-old woman with a history of infiltrative ductal carcinoma of the left breast who was hospitalised for neurological evaluation following repeated falls. During the course of her hospital stay, the patient's neurological state deteriorated rapidly, and onset of marked laboratory-documented inflammatory syndrome was observed, together with disseminated intravascular coagulation (DIC). At the same time, she developed orange-peel type skin lesions on her left breast followed by a rapidly-spreading livedoid infiltrated plaque on her side. The skin biopsy enabled a diagnosis to be made of intravascular lymphoma based on immunohistochemical demonstration of intravascular tumoral proliferation of lymphoid cells expressing CD45 marker and B-cell marker CD20, as well as Bcl2, MUM 1 and CD5. DISCUSSION: Intravascular B-cell lymphoma is a rare subtype of diffuse large B-cell lymphoma. It carries a poor prognosis and the clinical appearance varies extremely widely. The signs are primarily neurological and dermatological. Because of the varied nature of presentations as well as the rarity of the disease, diagnosis may be delayed, with worsening of the prognosis of the disease, which nevertheless requires rapid and aggressive management. However, the appearance of infiltrated and livedoid lesions on the trunk or limbs is evocative and skin biopsy constitutes a readily accessible diagnostic tool that offers rapid confirmation.
Asunto(s)
Neoplasias de la Mama/patología , Linfoma de Células B/patología , Recurrencia Local de Neoplasia/patología , Neoplasias Primarias Múltiples/patología , Piel/patología , Neoplasias Vasculares/patología , Anciano , Biopsia , Mama/irrigación sanguínea , Mama/patología , Diagnóstico Diferencial , Femenino , Humanos , Sensibilidad y Especificidad , Piel/irrigación sanguíneaAsunto(s)
Neoplasias Encefálicas/diagnóstico , Encefalitis/diagnóstico , Linfoma/diagnóstico , Rombencéfalo/patología , Biopsia , Neoplasias Encefálicas/complicaciones , Neoplasias Encefálicas/patología , Diagnóstico Diferencial , Encefalitis/etiología , Encefalitis/patología , Femenino , Humanos , Linfoma/complicaciones , Linfoma/patología , Imagen por Resonancia Magnética , Persona de Mediana EdadAsunto(s)
Histiocitosis de Células de Langerhans/diagnóstico , Dermatosis del Cuero Cabelludo/diagnóstico , Enfermedades Cutáneas Papuloescamosas/diagnóstico , Adulto , Amenorrea/etiología , Dermatitis Seborreica/diagnóstico , Diabetes Insípida Neurogénica/diagnóstico por imagen , Diabetes Insípida Neurogénica/etiología , Diabetes Insípida Neurogénica/patología , Diabetes Mellitus , Errores Diagnósticos , Femenino , Histiocitosis de Células de Langerhans/patología , Humanos , Hipopituitarismo/etiología , Hipotálamo/patología , Imagen por Resonancia Magnética , Neuroimagen , Hipófisis/patología , Dermatosis del Cuero Cabelludo/patología , Enfermedades Cutáneas Papuloescamosas/patologíaRESUMEN
â¢The optimal treatment of primary cutaneous angiosarcoma is considered to be surgical exision.â¢Concurrent chemo-radiotherapy is efficient in the treatment of primary cutaneous angiosarcoma.â¢The toxicity profile of this association is acceptable.