Kikuchi-Fujimoto necrotizing lymphadenitis associated with cutaneous lupus erythematosus: a case report

Histiocytic necrotizing lymphadenitis or Kikuchi-Fujimoto disease is a rare entity, and even more rarely, it is associated with other diseases. In a few cases, the condition is associated with cutaneous lupus erythematosus (CLE). Histiocytic necrotizing lymphadenitis predominantly affects young women, who present with solitary or multiple cervical lymphadenopathy accompanied by symptoms such as fever, weight loss, sweating, or, in exceptional cases, hepatosplenomegaly. Laboratory examinations show normal or nonspecific results. The disease is of unknown origin, although a viral origin has been suggested, with the suspected agents including Epstein-Barr virus, herpesvirus type 6, and cytomegalovirus (CMV). Although the first and most of the more recent cases have been reported in Oriental patients, the disease has a wide geographic distribution. The clinical evolution is favorable, with spontaneous remission in less than 4 months in almost all cases. We present a case of a 37-year-old woman from Peru who presented with cervical adenopathies on two occasions. Biopsy of a lymph node revealed a histopathologic picture compatible with Kikuchi-Fujimoto histiocytic necrotizing lymphadenitis. The adenopathy disappeared in a few months. A year later, she presented with a maculopapular rash in the nasal and malar regions. The results of the skin biopsy and immunofluorescence examination were compatible with chronic CLE. The results of the serology testing for CMV were positive. Treatment with chloroquine was initiated, with almost complete recovery by 5 months. No manifestations of systemic lupus erythematosus have occurred since. The epidemiologic, clinical, and anatomopathologic aspects as well as the differential diagnosis of this entity are reviewed.