Doença de Kikuchi-Fujimoto: apresentação de um caso

Abstract

The Kikuchi-Fujimoto disease (KFD) or histiocytic necrotizing lymphadenitis is a benign and self-limiting, rare disease, characterized by generalized lymphadenopathy, fever and leukopenia. It is an important differential diagnosis of lymphoproliferative disorders, autoimmune diseases, infectious diseases, and may in rare cases be associated with these. We report the case of a 24-year-old patient with fever, cervical and inguinal lymphadenopathy mucocutaneous injuries. Antinuclear antibodies were positive, blood count showed pancytopenia, negative anti-DNA. The inguinal lymph node biopsy revealed necrotizing histiocytic lymphadenitis. The patient presented clinical worsening of disease activity associated with sepsis, evolving with acute pericarditis and cardiac tamponade. Held cyclophosphamide pulse therapy, with substantial improvement of skin and systemic manifestations. The diagnosis of patients with KFD necessarily imp