Kikuchi–Fujimoto disease: a diagnostic dilemma

Abstract

Kikuchi-Fujimoto disease is a benign, self-limiting condition characterized by lymphadenopathy and fever. Its diagnosis can be challenging, as the disease must be differentiated from lymphoma and systemic lupus erythematosus. Patients can present with nonspecific cutaneous lesions. Adequate clinical and histopathologic correlation can aid in the timely diagnosis of Kikuchi's disease, thus preventing the use of unnecessary diagnostic procedures and inappropriate treatments.

A Mimic of Posttransplant Lymphoproliferative Disease Following Liver Transplant

“…However, severe cases of Kikuchi disease have been reported, with a mortality rate calculated to be 2.1%. 3 Cutaneous involvement, as occurred in this patient, often portends a more severe clinical course. Kikuchi disease in recipients of solid-organ transplants 1,4 and hematopoietic stem cell transplants 5 have rarely been reported, although the immunosuppressed state of transplant recipients may predispose to more severe Kikuchi disease, as the previously mentioned case series of Kikuchi disease in renal, liver, and pancreas recipients from the same donor resulted in recipient death.…”

“…2 The disease most commonly affects young Asian patients (< 40 years old), but there is debate about whether the disease has a marked female predisposition or instead has equal representation between the sexes. 3 Of note, our patient was born and raised in Korea, although she moved to the United States over 30 years ago. Kikuchi disease usually presents with lymphadenopathy (in particular cervical lymphadenopathy), fever, fatigue, and leukopenia.…”

“…2 Cutaneous manifestations, typically erythematous macules or papules, develop on the face, trunk, and/or upper extremities in 40% of patients with Kikuchi disease, similar to the rash that developed initially in our patient. 3 The lymphadenopathy and constitutional symptoms that accompany Kikuchi disease are indistinguishable from lymphoma, and misdiagnosis is thought to be common. However, pathologic evaluation of excised lymph nodes shows a unique pattern of histiocytic proliferation with patchy paracortical and cortical necrosis, abundant karyorrhectic debris, and a dearth of neutrophils.…”

“…However, pathologic evaluation of excised lymph nodes shows a unique pattern of histiocytic proliferation with patchy paracortical and cortical necrosis, abundant karyorrhectic debris, and a dearth of neutrophils. 3 The cause of Kikuchi disease is currently unknown, and both infectious (either viral or bacterial) and autoimmune causes have been advanced. 2,3 One case report documented transmission of Kikuchi disease to 3 separate organ transplant recipients who had received organs from a common donor, suggesting that some infectious trigger is likely.…”

“…3 The cause of Kikuchi disease is currently unknown, and both infectious (either viral or bacterial) and autoimmune causes have been advanced. 2,3 One case report documented transmission of Kikuchi disease to 3 separate organ transplant recipients who had received organs from a common donor, suggesting that some infectious trigger is likely. 4 Kikuchi disease is typically a relatively benign condition treated only with nonsteroidal antiinflammatory drugs and acetaminophen, and spontaneous resolution of symptoms commonly occurs within 1 to 4 months after diagnosis.…”