Kikuchi-Fujimoto disease type lymph node reaction with increased plasmacytoid dendritic cells may appear as a side effect following COVID-19 vaccination: Report of a case and literature review.

Subsequent to mass vaccination programs against COVID-19, diverse side effects have been described, both at the injection site, such as pain, redness and swelling, and systemic effects such as fatigue, headache, muscle or joint pain. On rare occasions, a lymphadenopathic syndrome may develop, raising the clinical suspicion of a lymphoproliferative disorder. We present the case of a 30-year-old woman who developed self-limiting left axillary lymphadenopathy following COVID-19 vaccination. To date, only seven similar cases with a complete clinicopathological description have been published, and fourteen cases have been notified to the European adverse events databases (Eudravigilance) in relationship with vaccination against COVID-19. It is important to be aware of this potential complication when a lymphadenopathic syndrome develops following vaccination, to avoid unnecessary treatment.

Kikuchi-Fujimoto-like lymphadenopathy following COVID-19 vaccine: diagnosis and management.

A woman in her mid 40s presented for breast imaging after 1 week of painful and enlarged right axillary lymphadenopathy. She denied history of fever, weight loss, night sweats fatigue, cat scratch or other trauma. She received the second dose of Pfizer COVID-19 vaccine 3 months previously on the contralateral arm. A mammogram demonstrated a single, asymmetric, large and dense right axillary lymph node. Ultrasound confirmed a 2.5 cm lymph node with cortical thickening of 0.6 cm. Ultrasound-guided core biopsy showed necrotising lymphadenitis with associated aggregates of histiocytes and plasmacytoid dendritic cells. Potential causes of necrotising adenitis including Bartonella, tuberculosis, Epstein-Barr Virus, herpes simplex virus, systemic lupus erythematosus and lymphoma were excluded. In the absence of any identifiable infectious or autoimmune causes, and given the temporal relatedness with vaccine administration, it was determined that the Kikuchi-Fujimoto-like necrotising lymphadenitis was likely secondary to the COVID-19 vaccine. To date, there has been no casual association made between the COVID-19 vaccine and KFD necrotising lymphadenitis.

Kikuchi-Fujimoto disease following SARS-CoV-2 vaccination.

A previously healthy Japanese woman in her 20s was admitted to our hospital with a 2-week history of fever (39.0°C) and a 1-week history of painful cervical lymphadenopathy. The day before fever onset, she had received her first Pfizer-BioNTech SARS-CoV-2 vaccine in her left arm. She had previously been treated with empirical antibiotics with no improvement. Physical examination revealed painful lymphadenopathy in both posterior cervical regions. CT showed symmetrical lymphadenopathies in the neck, supraclavicular, axillary and inguinal regions as well as hepatosplenomegaly. We suspected lymphoma and performed a lymph node biopsy in the right inguinal region, which revealed necrotising histiocytic lymphadenitis. The patient was, therefore, diagnosed with Kikuchi-Fujimoto disease (KFD). She improved after the corticosteroid therapy. This report highlights the importance of including KFD as a differential diagnosis of lymphadenopathy after SARS-CoV-2 vaccination. Additionally, lymph node biopsy is helpful for diagnosing KFD because it rules out other entities.

Clinicopathologic spectrum of necrotizing lymphadenitis.

BACKGROUND: Necrotizing lymphadenitis represents a group of diseases characterized by non-granulomatous inflammation and necrosis of the lymph node, caused by a variety of infective and inflammatory diseases, most common being Kikuchi-Fujimoto disease, acute Epstein Barr viral infection and systemic lupus erythematosis (1). OBJECTIVES: To study the morphological features in lymph nodes in cases of necrotizing lymphadenitis, to correlate them with specific etiological conditions.Materials and methods-58 cases of necrotizing lymphadenitis were reviewed and categorized into different etiological sub types, i.e. acute EBV lymphadenitis, lupus lymphadenitis and the rest as Kikuchis lymphadenitis. Morphological features studied were presence of vascular proliferation, periadenitis, foamy macrophage, neutrophil and plasma cell infiltrate. Clinical follow up was done. RESULTS: 62.2% of cases were Kikuchis lymphadenitis. Both lupus and Kikuchis had a female preponderance (78% and 62% respectively). Among the morphological parameters, plasma cell infiltration and vascular proliferation showed significant association with lupus lymphadenitis. Kikuchis and EBV lymphadenitis showed self-limiting course, with only 2 cases of Kikuchis developing recurrence .4 cases developed complications. All cases of lupus lymphadenitis needed long term therapy. CONCLUSION: Kikuchis lymphadenitis is the most common cause of necrotizing lymphadenitis, followed by lupus and acute EBV lyphadenitis.Young females were commonly affected in the first 2 groups. It is worthwhile to classify the cases of necrotizing lymphadenitis into etiological subgroups as the prognosis and treatment differ (2). Among the morphological features studied, plasma cell infiltrate and vascular proliferation were significantly associated with lupus lymphadenitis, hence can be used to predict etiology.

Kikuchi-Fujimoto Disease-Like Inflammatory Reaction in a Silicone Breast Implant Seroma.

INTRODUCTION: Kikuchi-Fujimoto disease (KFD) may have an autoimmune etiology and some cases have been associated with silicone breast implants. Cytomorphologic features of the disease have been well characterized by fine-needle aspiration of lymph nodes. They are so specific as to permit a precise cytologic diagnosis. Cytologic features have not been reported in fluid specimens. CASE: A 33-year-old female presented with a unilateral periprosthetic silicone breast seroma. The fluid was drained, and cytological analysis revealed numerous lymphocytes with no neutrophils, karyorrhectic nuclear debris, and peculiar histiocytes with eccentrically located nuclei showing a crescentic shape. Many of those histiocytes showed intracellular apoptotic debris. CONCLUSION: A Kikuchi disease-like inflammatory reaction is possible not only in axillary and cervical lymph nodes of patients with silicone breast implants but also in breast seromas. There is still not enough evidence to establish if there is an association between KFD and breast implants. A detailed cytologic examination of periprosthetic silicone breast seromas may help answer this question. In any case, pathologists must be aware of this possibility. Cytologic features are characteristic enough to permit differentiation from breast implant-associated anaplastic large-cell lymphoma.

A Mimic of Posttransplant Lymphoproliferative Disease Following Liver Transplant.

Generalized lymphadenopathy after organ transplant is a concerning finding, often indicating the devel-opment of lymphoma. We describe a 52-year-old liver transplant recipient who had clinical symptoms and imaging concerning for posttransplant lymphoproliferative disease. However, histologic evaluation of a lymph node biopsy revealed that the patient actually had a much rarer but relatively benign condition, Kikuchi-Fujimoto disease (histiocytic necrotizing lymphadenitis). We discuss the epidemiology, clinical symptoms, diagnosis, histologic features, and treatment of this uncommon mimic of posttransplant lymphoproliferative disease.

Kikuchi-Fujimoto disease triggered by Salmonella enteritidis in a child with concurrent auto-immune thyroiditis and papilloedema.

Kikuchi-Fujimoto disease (KFD) is a histiocytic necrotising lymphadenitis characterised by painful cervical lymphadenopathy, fever, malaise and weight loss. Infections, auto-immune pathogenesis and a genetic association have been implicated. A 12-year-old boy presented with a 1-month history of fever, abdominal pain, constipation and weight loss, and a painful lymph node was detected in the right axilla. Chest CT demonstrated multiple lymph nodes, especially in the left mediastinum. Salmonella enteritidis group D was detected in a blood culture and he was treated with ceftriaxone, followed by meropenem. An axillary lymph node biopsy demonstrated necrotising histiocytic lymphadenitis and KFD was diagnosed. He was discharged 35 days after admission. He was re-admitted 3 weeks later with recurrence of symptoms and headache and was found to have papilloedema of the left eye and auto-immune thyroiditis. Intravenous immunoglobulin (IVIG) 400 mg/kg/day was administered for 5 days. The fever and papilloedema slowly resolved and, subsequently, the thyroiditis, and he has remained well on follow-up. This is the first report of an association of S. enteritidis infection and papilloedema with KFD. IVIG may be required in prolonged or recurrent cases and in those with an auto-immune association.

Facial and limb angioedema with parotitis and Kikuchi-like necrotizing lymphadenitis preceding neuropsychiatric systemic lupus erythematosus in a young African American male.

Angioedema has been observed in a few cases secondary to systemic lupus erythematosus (SLE). Herein, we report a rare case where a young healthy male initially presented with angioedema, lymphadenopathy and parotitis and later on developed neuropsychiatric manifestations at the very onset of his SLE disease. This case illustrates the importance of prompt clinical consideration of lupus with unusual and atypical preceding manifestations.

Epidemiology and Characteristics of Kikuchi-Fujimoto Disease in the African-Descent Population of Martinique, French West Indies.

OBJECTIVE: To provide an epidemiologic description of Kikuchi-Fujimoto disease (KFD), and to describe its relationship with systemic lupus erythematosus (SLE) in a population of sub-Saharan origin. METHODS: Patients were retrospectively included on the basis of lymph node histology compatible with KFD reported in Martinique from 1991 until 2013. In order to describe the characteristics of the disease in a larger cohort, we subsequently included more patients of Afro-Caribbean origin from Guadeloupe and French Guiana. RESULTS: In Martinique, mean annual incidence between 1991 and 2013 was 2.78 cases for 1 million inhabitants (95% confidence interval 1.73-3.93). A total of 36 Afro-Caribbean patients from the 3 French American regions were included. Mean age was 30.5 years (range 5-59 years) and the female:male ratio was 3:1. The main characteristics were cervical adenopathies (88.8%), fever (83.3%), asthenia (73.0%), weight loss (64.4%), and recurrence in 33.3%. KFD was associated with lupus (n = 9 for SLE, n = 2 for cutaneous lupus) in 36.6% (11 of 30). CONCLUSION: We report the first epidemiologic description of KFD in a population of sub-Saharan origin. According to our data, this disease is present in the black African diaspora and is strongly associated with autoimmune diseases, particularly lupus.

Kikuchi Disease-Like Inflammatory Pattern in Cutaneous Inflammatory Infiltrates Without Lymph Node Involvement: A New Clue for the Diagnosis of Lupus?

Kikuchi-Fujimoto disease (KFD) is a rare and benign disorder that usually occurs in young adults with enlarged lymph nodes containing infiltrate of cytotoxic T cells and nuclear debris. It can be a manifestation of systemic lupus erythematosus (SLE) although the strength of this association has varied among studies. Although specific KFD cutaneous lesions are well described, pure cutaneous lesions have never been reported. We studied a series of patients prospectively entered into a database between 2007 and 2014 with skin biopsies showing diffuse or localized inflammatory infiltrates reminiscent of cutaneous KFD, without lymph-node-related KFD. We called these skin lesions "Kikuchi disease-like inflammatory pattern" (KLIP). Twenty-nine patients, whose median age was 49 years at the time of skin biopsy, were selected and retrospectively analyzed using standardized clinical and histology charts. In skin biopsies, KLIP was localized to restricted areas within the inflammatory infiltrate (17%) or diffuse (83%), and was the only histological finding (45%) or accompanied interface dermatitis with or without dermal mucinosis (55%). Clinical dermatological findings varied widely. A definite diagnosis could be established for 24 patients: 75% had connective tissue diseases or vasculitis, mainly cutaneous lupus erythematosus (CLE) (n = 16, 67%), including 5 SLE with satisfying American College of Rheumatology criteria; 3 of the remaining patients had malignant hemopathies. CLE patients were mostly young females with acute (n = 5), subacute (n = 4), or chronic CLE (n = 6) or lupus tumidus (n = 1). Two were classified as having anti-tumor necrosis factor-alpha-induced lupus. Because two-thirds of these patients were finally diagnosed with CLE, we think that KLIP may represent a new histopathological clue for the diagnosis of lupus based on skin biopsy, requiring clinical-immunological comparison to make the correct diagnosis. KLIP should not be considered a variant of classical KFD, but rather as an elementary pattern of cutaneous inflammation, that might be the expression of the same cytotoxic process within skin infiltrates as that involved in KFD. This lesion might reflect a particular T-cell-mediated autoimmune process directed against mononuclear cells within cutaneous lupus infiltrates.

Kikuchi-Fujimoto's disease associated with systemic lupus erythematous: difficult case report and literature review.

Kikuchi-Fujimoto's disease (KFD), or histiocytic necrotizing lymphadenitis, is a benign and self-limiting disease of unknown aetiology. KFD tends to affect a young population under 30 years of age and predominantly females. KFD is a rare pathology and its association with systemic lupus erythematosus (SLE) is not frequent. Herein, we present the case of a male Italian patient with SLE in association with KFD with 5 years of follow-up, where a differential diagnosis from infection or lymphoproliferative disease was problematic.

Enfermedad de Kikuchi-Fujimoto / Kikuchi-Fujimoto’s disease

La Enfermedad de Kikuchi-Fujimoto, también llamada Linfadenitis Histiocítica Necrosante, es un sindrome benigno y autolimitado descubierto por primera vez en 1972 por Kikuchi y por Fujimoto, dos patólogos japoneses. Es una enfermedad principalmente descrita en mujeres jóvenes hasta los 40 años de edad que se ha encontrado en todos los grupos étnicos y que puede presentarse en una proporción menor en hombres. La etiología es desconocida, aunque se ha asociado a ciertos microorganismos, y son las infecciones junto con el linfoma los principales diagnósticos diferenciales de esta enfermedad, caracterizada por linfadenopatía cervical con dolor, fiebre leve y síntomas sistémicos. El estudio histopatológico de los ganglios linfáticos permite diferenciar esta enfermedad de otras más graves, aunque es indistinguible histológicamente del Lupus Eritematoso Sistémico con el cual puede presentarse concomitantemente.

Kikuchi-Fujimoto’s disease, also called Histiocytic Necrotizing Lymphadenitis is a benign, self-limited syndrome. It is a disease mainly described in young women under 40 years of age, it has been found in all ethnic groups and may occur in a lesser extent in men. The etiology is not known, although it has been associated with some microorganisms. The differential diagnoses of this disease are infections and lymphoma. This disease is, characterized by cervical lymphadenopathy with pain, mildfever and systemic symptoms. Histopathological study of lymphnodes differentiate this disease from others more serious, although histologically it’s indistinguishable of Systemic Lupus Erythematosus with which can occur concomitantly.

Kikuchi-Fujimoto disease complicated by peripheral neuropathy.

Kikuchi-Fujimoto disease is a necrotizing lymphadenitis, mainly characterized by lymphadenopathy, fever, hepatosplenomegaly, nocturnal sweats, myalgia, weight loss, and arthralgia. Its diagnosis is most often based on lymph node biopsy. Differential diagnoses with several other diseases, e.g., malignant lymphoma, necrotizing lymphadenitis, and infective lymphadenopathies, may be challenging. Neurologic involvement is rarely reported in patients diagnosed with Kikuchi-Fujimoto disease. In this subset of patients, the great majority manifest signs involving the central nervous system. We present a 14-year-old boy with a severe form of Kikuchi-Fujimoto disease, complicated by peripheral neuropathy. This patient is interesting for both his age and his peculiar complication.

Kikuchi-Fujimoto disease following vaccination against human papilloma virus infection and Japanese encephalitis.

Kikuchi-Fujimoto disease (KFD) is a benign and self-limited syndrome, characterized by regional lymphadenopathy and fever. Although the pathogenesis of KFD remains unclear, infectious agents, autoimmune causes, and physicochemical factors have been suggested as triggers. However, KFD following vaccination has never been reported. We present a 14-year-old girl who suffered from fever and cervical lymph node swelling following simultaneous administration of human papilloma virus vaccine and Japanese encephalitis virus vaccine. The patient was diagnosed with KFD based on the histopathologic findings of a lymph node biopsy, and her fever and swelling resolved with oral corticosteroid therapy. Although the exact pathogenesis of the development of KFD following immunization remains unknown, this should be added to the list of potential triggers or factors associated with the development of KFD.