Systemic Kikuchi-Fujimoto disease bordering lupus lymphadenitis: A fresh look?

Kikuchi-Fujimoto disease preceded by lupus erythematosus panniculitis: do these findings together herald the onset of systemic lupus erythematosus?

“…Yet, many cases of lupus lymphadenitis, including the present example, are devoid of these distinguishing features [3,21,22]. Extensive effacement of nodal architecture, which was identified in the present case and contrasts with the patchy, delineated foci of paracortical necrosis often seen in KFD, is also more common in lupus [4]. However, the amount and localization of necrosis can vary widely in KFD [12].…”

“…Lupus lymphadenitis and KFD share the remainder of these histologic findings. However, the presence of neutrophils, hematoxylin bodies, and the Azzopardi phenomenon would favor lupus [4]. Yet, many cases of lupus lymphadenitis, including the present example, are devoid of these distinguishing features [3,21,22].…”

“…Kikuchi-Fujimoto disease was described almost simultaneously by Kikuchi and Fujimoto in 1972 [9,10]. Although it has been documented in patients of various racial and ethnicities, there is a noted prevalence among women of east Asian ancestry with a typical disease onset in the early twenties to mid-thirties [4]. Kikuchi-Fujimoto disease most frequently involves the cervical lymph nodes, although generalized lymphadenopathy and involvement of retroperitoneal, peritoneal, mediastinal, inguinal, axillary, supraclavicular, and intraparotid nodes has been described [4,[11][12][13][14].…”

“…Hepatosplenomegaly has also been reported [13]. Patients usually describe low-grade fever, malaise, and night sweats [4]. Laboratory abnormalities are nonspecific and can include anemia, leukopenia,…”

“…This is important because KFD is considered a benign and self-limited disorder. In contrast, SLE can be life-threatening, requiring coordination of care among a multidisciplinary network of healthcare providers [4]. Therefore, the diagnosis of KFD should be made with the caveat that histologic findings can be indistinguishable from those of lupus and patients should be monitored for the dermatologic and…”