Kikuchi disease,  also called histiocytic necrotizing  lymphadenitis or Kikuchi-Fujimoto disease, was described for the first time in 1972 in Japan. It is a benign, self-limiting disease (it resolves without specific treatment) (usually within 2 months but recurrences are reported)  of unknown etiology because no causal link has been established. Investigators have postulated a viral triggering ( Epstein-Barr virus, Parvovirus B 19?) or an autoimmune pathogenesis. It is more common in Asia (among Koreans), predominantly young women.

It presents with swelling of painful cervical lymph nodes, usually at a single site (but can be affected multiple lymph node regions, such as axillary, supraclavicular, mediastinal, inguinal regions), fever, myalgia, leucopenia (especially in those with prolonged fever).  Liver and spleen may be involved.

Skin rashes of nonspecific nature are reported:  erythematous macules, papules, papulopustular or papulonodular eruption, plaques or ulcers localized on the trunk, upper extremities, face. In small cases lip or eyelid edema.

Reported constitutional symptoms: diarrhea, nausea, vomiting, sweats, abdominal and chest pain. The biopsy of affected lymph nodes shows a typical histopathology: reactive hyperplasia with expansion of the paracortex, rich in apoptotic plasmacytoid monocytes (CD123+, CD68+, BCL2–) and phagocytic histiocytes.

Because Kikuchi disease is self-limited, medical treatment can be helpful in cases that do not abate or in recurrences (systemic steroids, naproxen, minocycline, intravenous immunoglobulin). Differential diagnosis is with Hodgkin disease, lymphoma, systemic lupus erythematosus.