Kikuchi-Fujimoto Disease

Abstract

Kikuchi-Fujimoto disease (KFD) is a rare cause of lymphadenopathy, most often cervical. It has been mainly described in Asia. There are few data available on this disease in Europe. We conducted this retrospective, observational, multicenter study to describe KFD in France and to determine the characteristics of severe forms of the disease and forms associated with systemic lupus erythematosus (SLE). We included 91 cases of KFD, diagnosed between January 1989 and January 2011 in 13 French hospital centers (median age, 30 ± 10.4 yr; 77% female). The ethnic origins of the patients were European (33%), Afro-Caribbean (32%), North African (15.4%), and Asian (13%). Eighteen patients had a history of systemic disease, including 11 with SLE. Lymph node involvement was cervical (90%), often in the context of polyadenopathy (52%), and it was associated with hepatomegaly and splenomegaly in 14.8% of cases. Deeper sites of involvement were noted in 18% of cases. Constitutional signs consisted mainly of fever (67%), asthenia (74.4%), and weight loss (51.2%). Other manifestations included skin rash (32.9%), arthromyalgia (34.1%), 2 cases of aseptic meningitis, and 3 cases of hemophagocytic lymphohistiocytosis. Biological signs included lymphocytopenia (63.8%) and increase of acute phase reactants (56.4%). Antinuclear antibodies (ANAs) and anti-DNA antibodies were present in 45.2% and 18% of the patients sampled, respectively. Concomitant viral infection was detected in 8 patients (8.8%). Systemic corticosteroids were prescribed in 32% of cases, hydroxychloroquine in 17.6%, and intravenous immunoglobulin in 3 patients. The disease course was always favorable. Recurrence was observed in 21% of cases. In the 33 patients with ANA at diagnosis, SLE was known in 11 patients, diagnosed concomitantly in 10 cases and in the year following diagnosis in 2 cases; 6 patients did not have SLE, and 4 patients were lost to follow-up (median follow-up, 19 mo; range, 3–39 mo). The presence of weight loss, arthralgia, skin lesions, and ANA was associated with the development of SLE (p < 0.05). Male sex and lymphopenia were associated with severe forms of KFD (p < 0.05). KFD can occur in all populations, irrespective of ethnic origin. Deep forms are common. An association with SLE should be investigated. A prospective study is required to determine the risk factors for the development of SLE.

A Case of Kikuchi-Fujimoto Disease Associated with Erosive Lichen Planus

“…It usually occurs in adults below the age of 40, although it can affect any age group. Both genders can be affected, with most reports showing a female predominance [2,5,8].…”

“…It is a rare, benign, self-limiting condition with a good prognosis [2]. Although reported worldwide in a variety of ethnic groups, KFD classically affects young women with the highest prevalence being in Asia [3][4][5].…”

“…The most common clinical features include cervical lymphadenopathy, fever, and cutaneous eruptions with or without systemic symptoms [6]. The most common cutaneous manifestations of KFD are nonspecific skin rashes, erythematous maculopapular lesions, nodules, erythematous papules, indurated erythematous lesions, and erythema multiforme with areas on the face and upper trunk most frequently affected [2,5].…”

Kikuchi disease – a rare cause of pyrexia of unknown origin

“…Kikuchi disease was identified in 1972, in Japan, by the pathologists Kikuchi and Fujimoto, independently. It mainly affects young Asian adults with a female predominance [2]. It is a benign mimic of serious conditions such as systemic lupus erythematosus, lymphoma and tuberculous lymphadenitis which need careful exclusion prior to diagnosis as a Kikuchi disease [1,2,3].…”

“…It mainly affects young Asian adults with a female predominance [2]. It is a benign mimic of serious conditions such as systemic lupus erythematosus, lymphoma and tuberculous lymphadenitis which need careful exclusion prior to diagnosis as a Kikuchi disease [1,2,3]. Since there are no immunohistochemical markers for Kikuchi disease available at present, diagnosis is made by excluding other serious conditions.…”