Kikuchi–Fujimoto and Kimura diseases: the selected, rare causes of neck lymphadenopathy

Abstract

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a benign and self-limiting disease typically characterized by the enlargement of regional lymph nodes and accompanied by fever. KFD affects predominantly young adult females of Asian origin and is rarely seen in European countries, where it may cause diagnostic difficulties. Kimura disease is a rare and benign chronic inflammatory soft tissue disorder of unknown origin, characterized by a triad of painless subcutaneous masses in the head or neck region accompanied by regional lymphadenopathy, blood and tissue eosinophilia, and markedly elevated serum immunoglobulin E levels. Although most cases of Kimura disease have originated in China, Japan or Southeast Asia, there have been sporadic case reports from Europe and America. Herein, we review in detail the clinical presentations, complications and current concepts in the pathogenesis, diagnosis and treatment of these diseases.

Kimura’s Disease: A Case Report in a Child

“…Some evidence has indicated that the interaction between T H 1 and T H 2 lymhocytes may result in the excessive production of eosinophilotrophic cytokines, such as interleukin 4. Persistent antigenic stimulation from insect bites, parasitic infestation, candidal infection, or viral infection may cause the activation of this cytokine pathway, but further investigation is needed [3,4]. Kimura's disease involves the skin, lymph nodes, and salivary glands and is reported to be associated with nephrotic syndrome in approximately 15-19% of cases.…”

Kikuchi’s disease in a young Scandinavian woman

“…Cellular in iltrates consist of CD68+/CD123+ plasmacytoid histiocytes and transformed lymphocytes, mainly CD8+ T-lymphocytes expressing perforin involved in provoking apoptosis in target cells. Tubuloreticular structures in histiocytes, activated T cells and endothelial cells resemble those seen in SLE and viral disease [5]. However in SLE lymphadenitis neutrophils, plasma cells and haematoxylin bodies are often present [2].…”

“…The unspeci ic symptoms and the rarity of the illness means that there are many other differential diagnoses to consider, e.g. lymphoma, tuberculosis, metastatic disease, infectious mononucleosis, systemic lupus erythematous (SLE) or cat scratch disease [1,[5][6][7][8].…”

“…It is believed that an unknown infectious trigger incites an immune response of T-lymphocytes and histiocytes in which the cellular destruction seen is apoptosis brought on by CD8 T-lymphocytes. One study showed an oligoclonal pattern of T-cell receptor antigen rearrangement, thus indicating a benign reaction rather than clonal T-cell malignancy [5]. A variety of infectious agents (Brucella, Yersenia, toxoplasmosis, Ebstein-Barr virus (EBV), human herpes virus 6 (HHV6), hepatitis B virus (HBV), cytomegalovirus (CMV)) are suspected, but blood cultures, polymerase chain reaction (PCR), serological studies and hydridization technique examinations are usually negative [1,2,4].…”

“…Some recommend non-steroidal anti-in lammatory drugs (NSAID) for symptom relief and in more aggressive cases systemic corticosteroids [5,6,11]. Symptoms most frequently resolve within 6 months [1,9], but has been reported to last up to 37 months [12].…”