Cutaneous Kikuchi Disease

Updated: Feb 10, 2016
  • Author: Noah S Scheinfeld, JD, MD, FAAD; Chief Editor: Dirk M Elston, MD  more...
  • Print
Overview

Background

Kikuchi disease (KD) was reported for the first time in 1972 in Japan by Kikuchi and by Fujimoto and his colleagues. Kikuchi disease is an idiopathic, self-limited necrotizing lymphadenitis. Kikuchi disease commonly presents with cervical lymphadenopathy accompanied by fever, myalgia, neutropenia, and rash in one third of cases. Involvement of extranodal sites is unusual but is described, especially in Asia where Kikuchi disease is more common than in North America or Europe. It is most common in young women.

Kikuchi disease can clinically and histologically mimic Hodgkin disease or high-grade lymphoma. It can also resemble the lymphadenitis of systemic lupus erythematosus (SLE).

Related Medscape articles include Hodgkin Lymphoma, Pediatric Hodgkin Lymphoma, High-Grade Malignant Immunoblastic Lymphoma, Lymphadenitis, and Systemic Lupus Erythematosus (SLE).

Next:

Pathophysiology

Investigators have postulated that apoptosis plays an important part in the pathogenesis of Kikuchi disease. They believe that cytotoxic T lymphocytes are apoptotic effectors as well as target cells and that histiocytes could possibly enhance apoptosis in Kikuchi disease. The trigger of this process may have either a viral pathogenesis or an autoimmune pathogenesis.

Plasmacytoid monocytes have been suggested to play a role in the pathogenesis of Kikuchi disease via a cell-mediated cytotoxic immune response.

Kikuchi disease has been reported in 2 nontwin sisters with HLA-identical phenotypes, who presented 10 years apart.

Zhang et al [1] described an association between parvovirus B19 and Kikuchi-Fujimoto disease. Specifically, from one case of Kikuchi disease, they reported that the in situ hybridization and immunohistochemistry double-staining assay results revealed that parvovirus B19–infected cells were mainly lymphocytes and a small number of histiocytes.

Previous
Next:

Epidemiology

Frequency

United States

Kikuchi disease is rare.

International

Kikuchi disease is more prevalent among Asians and is a relatively common disorder among Koreans.

In Taiwan, Chuang et al [2] studied 64 patients younger than 18 years with pathologic proof of Kikuchi disease. In this group of 35 males and 29 females, the age range was 2-18 years, with a median age of 16.63 years for the males.

The female patients had cervical lymphadenopathy, and 1 had generalized lymphadenopathy. In 50% of patients, the lymph nodes were painful, tender, or both. In 82.5% of patients, the lymphadenopathy was unilateral. In 32.8% of patients, the lymphadenopathy was associated with fever.

Rash, hepatomegaly, or weight loss occurred in less than one third of the patients. Leukopenia was present, and 3% had leukocytosis. Mild liver dysfunction was present in approximately 25% of patients. Most patients had normal virologic or immunologic study results. Patients with prolonged fever were more likely to have leukopenia (P < .05). All 64 patients had remissions of Kikuchi disease, but 1 developed SLE 5 years later and another had a vasculitis syndrome 2 years later.

Rau and Kini [3] reported 20 patients with Kikuchi disease from an Indian referral center, one of whom had skin involvement.

Race

This disease affects Asians more than other races, but it has been reported worldwide.

Sex

Females are more commonly affected than males, with a female-to-male ratio of 4:1.

Age

Kikuchi disease affects a wide age range from 10 months to 75 years, with a mean patient age in the third decade.

Previous
Next:

Prognosis

Kikuchi disease is a self-limited condition that can recur in about 3% of cases. Therefore, the prognosis is excellent.

Besides the pain associated with the condition, it has minimal mortality and morbidity. If Kikuchi disease occurs in patients who have undergone transplantation, it can be fatal. Kikuchi disease can evolve into lupus, in which case morbidity and mortality are substantially increased.

Previous
Next:

Patient Education

Patients must know that they have a benign and self-limited condition.

Previous