• Main Authors: Paula Maio, Candida Fernandes, Ana Afonso, Fernanda Sachse, José Cabeçadas, Jorge Cardoso
• Format: Article
• Language: English
• Published: Sociedade Brasileira de Dermatologia 2013-02-01
• Series: Anais Brasileiros de Dermatologia
• Subjects: Células dendríticas; Leucemia; Neoplasias; Dendritic cells; Leukemia; Neoplasms
• Online Access: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962013000100131
• ISSN: 0365-0596; 1806-4841

Blastic plasmacytoid dendritic cell tumor is a rare, highly aggressive systemic neoplasm for which effective therapies have not yet been established. We describe a 73-year-old man with multiple nodules and patches emerging on the trunk and limbs. Lesional skin biopsy revealed a plasmacytoid dendritic cell tumor with dense dermal infiltrate of tumor cells with blastoid features. No apparent systemic involvement was identified in the initial stage. The patient was treated with prednisone daily, with notorious improvement of the skin lesions, although no complete remission was obtained. During the six-month follow-up period, no disease progression was documented, but fatal systemic progression occurred after that period of time.<br>A leucemia de c&#233;lulas dendr&#237;ticas plasmocit&#243;ides bl&#225;sticas &#233; uma entidade de classifica&#231;&#227;o recente. Descrevese o caso de um homem de 73 anos com dermatose envolvendo manchas, placas infiltradas e n&#243;dulos eritematosos na face anterior do tronco, no dorso e nos membros. A biopsia cut&#226;nea revelou presen&#231;a de infiltra&#231;&#227;o difusa por c&#233;lulas linf&#243;ides de tamanho intermedi&#225;rio. A imunocitoqu&#237;mica permitiu o diagn&#243;stico de neoplasia de c&#233;lulas dendr&#237;ticas plasmocit&#243;ides bl&#225;sticas. O estadiamento n&#227;o revelou envolvimento sist&#234;mico na &#233;poca do diagn&#243;stico. O doente iniciou a terap&#234;utica com prednisolona e apresentou remiss&#227;o inicial das les&#245;es cut&#226;neas. Posteriormente, observou-se progress&#227;o sist&#234;mica da doen&#231;a, e o doente veio a falecer.