Enigmatic Kikuchi-Fujimoto Disease: A Comprehensive Review

Xavier Bosch, MD; Antonio Guilabert, MD; Rosa Miquel, MD; Elias Campo, MD

Disclosures

Am J Clin Pathol. 2004;122(1) 

In This Article

Unusual Features and Associated Conditions

Involvement of extranodal sites by KFD is uncommon, but skin and bone marrow involvement and liver dysfunction have been reported.[19,56,57,58]

It is worth mentioning that 6 of 7 initially reported cases of cutaneous involvement occurred in males and that the facial skin was the most common localization. The patients had had a disease with a more severe clinical course, yet all recovered without complications. Skin lesions in KFD seem to be nonspecific. A wide variety of dermatological patterns have been observed, including rashes; nodules; erythematous, crusted papules; scattered, indurated, erythematous lesions; erythema multiforme; and erythematous maculopapular eruptions, all mainly affecting the face and upper body.[59,60,61,62,63] A recent article described a patient with papulopustular whole-body skin involvement.[64] Oral ulcers and lip edema with desquamation and erosions have been reported anecdotally.[19,65] Finally, a patient was described whose recalcitrant psoriasis cleared spontaneously during the clinical course of KFD.[66]

A few patients have had generalized lymphadenopathy and hepatosplenomegaly as the initial manifestations of KFD. KFD also has been reported as a cause of prolonged fever of unknown origin.[67,68,69] It has been reported rarely in HIV-positive patients[70,71] and in association with the human T-cell leukemia/lymphoma (lymphotropic) virus type 1 (HTLV-1). One patient with HTLV-1 had a recurrent clinical form of KFD along with aseptic meningitis.[34,35] Charalabopoulos et al[72] described a case of KFD associated with brucellosis. A recent article from Thailand suggested an association between Mycobacterium szulgai lymphadenitis and KFD on the basis of the coexistence of characteristic histologic features of KFD in lymph nodes and a positive culture.[73] On the other hand, SLE has developed in some patients thought to have true KFD, suggesting to some investigators that KFD could be an incomplete form of an autoimmune condition. The patients, however, may have had SLE from the beginning, taking into account the histopathologic considerations discussed later.[74,75,76,77] The diagnosis of KFD can precede, postdate, or coincide with the diagnosis of SLE.[75] With regard to this topic, in 2003, Hu and coworkers[78] reported a clinicopathologic analysis of 18 cases of KFD plus SLE and tried to clarify the relationship between the conditions. They concluded that KFD is not related to SLE and that the KFD-like lymphadenitis coexisting with SLE should be regarded as a lupus lymphadenitis on the basis of several histologic criteria. Nevertheless, 6 cases of pre-SLE or post-SLE necrotizing lymphadenitis were found to be true KFD.[78]

In addition to SLE, other autoimmune conditions and manifestations such as antiphospholipid syndrome, polymyositis, systemic juvenile idiopathic arthritis, bilateral uveitis, arthritis, cutaneous necrotizing vasculitis, and pulmonary hemorrhage have been linked to KFD.[79,80,81,82,83,84,85] Whether these patients had true KFD or another autoimmune necrotizing lymphadenitis such as that associated with SLE remains unproven. Furthermore, some fatal cases, which had been reported as KFD, were found to be related to potentially life-threatening autoimmune conditions.[85,86]

There have been other reports of unusual features of KFD, including parotid gland involvement,[87,88] thyroiditis,[88] carcinoma,[89] and diffuse large B-cell lymphoma.[90] Some cases of KFD have been associated with the hemophagocytic syndrome.[36,81,91,92] A review by Kelly and coworkers[91] on this association concluded that the diseases should be considered as a continuum rather than as separate entities.

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