Necrotizing histiocytic lymphadenitis (NHL) or Kikuchi-Fujimoto disease is an infrequent seen clinicopathologic entity that affects most frequently young women and individuals of eastern races, and that is characterized by fever and adenopathies (basically cervical) and systemic disease even though occasional patients present extranodal disease. We present two young women with NHL and cutaneous affectation, and with lymphocytic meningitis in one of the patients. We discuss the etiology of NHL, the relation of NHL with collagen-vascular diseases (basically systemic erythematous lupus) the problem of the pathologic diagnosis, and the therapeutic possibilities in these patients.