Kikuchi—Fujimoto's Necrotizing Lymphadenitis in Association with Discoid Lupus Erthematosus: A Case Report

2004 ◽  
Vol 8 (6) ◽  
pp. 442-445
Author(s):  
Shane G. Silver ◽  
H. Chih-Ho Hong ◽  
Patricia T. Ting ◽  
Nigel J. Ball

Background: Kikuchi–Fujimoto's necrotizing lymphadenitis (KFNL) is a rare, benign, self-limited condition characterized by constitutional symptoms, lymphadenopathy, and skin lesions. Objective: We report a case of KFNL in a 43-year-old East Indian woman with a ten-year history of discoid lupus erythematosus (DLE) of the scalp and a three-month history of a erythematous plaque on the left nasal bridge, cervical lymphadenopathy, and fever. Skin biopsy samples were taken from the face and lymph node. Results: Histopathological examination of the skin revealed a mixed infiltrate of inflammatory cells, nuclear dust, and histiocytes phagocytosing nuclear debris in the reticular dermis. The lymph node showed interfollicular liquefactive necrosis, immunoblasts, and a similar cellular infiltrate as the skin. The non-necrotic areas demonstrated follicular hyperplasia. These pathological changes are associated with a diagnosis of KFNL. Conclusions: KFNL is reported in association with systemic lupus erythematosus, but only two other cases of systemic KFNL in association with DLE exist in the literature. This case is unique in that the patient presented with cutaneous and systemic KFNL in the setting of longstanding DLE.

F1000Research ◽  
2016 ◽  
Vol 5 ◽  
pp. 1407
Author(s):  
Arvind P. Ganpule ◽  
Jaspreet Singh Chabra ◽  
Abhishek G. Singh ◽  
Gopal R. Tak ◽  
Shailesh Soni ◽  
...  

Kikuchi-Fujimoto disease (KFD) is an extremely rare disease with a worldwide distribution and higher prevalence in Asians. It is a benign and self-limiting disorder, characterized by regional cervical lymphadenopathy accompanied with mild fever and night sweats. Lymph node histopathology is diagnostic and treating physicians should be aware of this entity as it may mimic other systemic diseases like systemic lupus erythematosus, tuberculosis, malignant lymphoma, and more rarely adenocarcinoma. Key features on lymph node biopsy are fragmentation, necrosis and karyorrhexis. Treatment includes symptomatic care, analgesics-antipyretics, corticosteroids and spontaneous recovery occurs in 1 to 4 months. We report a case of adult polycystic kidney disease (ADPKD) with end stage renal disease and episodes of fever and cervical lymphadenopathy. The infectious screen was negative and on extensive workup, the patient was found to have histiocytic-necrotizing lymphadenitis, which clinched the diagnosis of KFD.


Author(s):  
Ahmed A Mohamad ◽  
Muhammed Zahid ◽  
Adeel Ahmad Khan ◽  
Belal Alani ◽  
Mustafa Khalil ◽  
...  

Introduction: Kikuchi-Fujimoto (KF) disease is a rare and self-limiting disorder of unknown aetiology characterized by cervical lymphadenopathy (CLN) and fever. The pathophysiology remains unclear and may be triggered by an infectious agent leading to a self-limiting autoimmune process. There are no confirmatory laboratory tests and lymph node biopsy is required to differentiate KF disease from other serious conditions. Materials and methods: We report 11 cases of KF disease diagnosed at Hamad General Hospital, Qatar, between 2006 to 2016. The diagnosis is based on clinical presentation, investigations and histopathological examination of lymph nodes. Results: All patients had painful neck swelling (average duration of 2.9 weeks) and 10 had fever (average duration of 3.2 weeks). Five patients developed mild leucopenia which resolved completely. HIV and tuberculosis (TB) screening including sputum for AFB, a PPD skin test and chest x-ray was done for all patients and came back negative. Autoimmune screening was done for all patients and excluded any rheumatological disease. Ultrasound and CT of the neck confirmed cervical lymphadenopathy. Except for hepatomegaly in one patient, CT scans of the chest and abdomen were negative for any lymphadenopathy or organomegaly (performed in seven patients). Diagnosis was confirmed on lymph node excision biopsy. Histopathological examination showed findings consistent with the diagnosis of histiocytic necrotizing lymphadenitis (KF disease). Conclusion: KF disease should be kept in mind for patients presenting with fever and CLN. Lymphoma, TB and autoimmune diseases like systemic lupus erythematosus should be excluded in such patients.


2019 ◽  
Vol 57 (1) ◽  
pp. 72-77
Author(s):  
Taro Horino ◽  
Osamu Ichii ◽  
Yoshio Terada

Abstract Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare, benign, self-limiting disease characterized by cervical lymphadenopathy and fever. Since KFD was first reported in 1972, the validity of this clinical entity has been controversial and its aetiology remains unknown. Herein, we report a case of a patient with KFD, which was believed to be associated with systemic lupus erythematosus.


2021 ◽  
Vol 8 (9) ◽  
pp. 1602
Author(s):  
Sushil Singla ◽  
Mohitesh Kumar ◽  
Vinod Kumar Jat ◽  
Deepika Parwan

Kikuchi-Fujimoto disease (KFD) is a rare benign condition also called histiocytic necrotizing lymphadenitis, which typically presented as fever with cervical lymphadenopathy in previously healthy individual. We presented a case of 11 year old boy with fever and cervical lymphadenopathy since 2 months. Lymph node biopsy was performed which suggested of KFD and was treated symptomatically. KFD incidence is rare but clinicians should be alert if young patient comes with fever and cervical lymphadenopathy to lower the chance of unwanted laboratory test and harmful treatment. 


2018 ◽  
pp. bcr-2018-226457
Author(s):  
Roman Zuckerman ◽  
Louise Damiani ◽  
Hashem A Ayyad ◽  
Deborah R Alpert

We describe a 50-year-old woman with a history of thyroid cancer who presented with bilateral cervical and submandibular lymphadenopathy, low-grade fevers, and increasing fatigue. The patient underwent lymph node fine-needle aspiration, which showed no evidence of metastatic or lymphoproliferative disease. This procedure was complicated by a parapharyngeal abscess and cellulitis. She was treated unsuccessfully with various courses of antibiotics, but briefly responded to short courses of steroids. As her cervical lymphadenopathy returned, she underwent an excisional lymph node biopsy, which demonstrated caseating granulomatous lymphadenitis. Extensive review of systems, physical examination, laboratory and imaging studies demonstrated no evidence of malignancy, infection or systemic lupus erythematosus . Our patient was clinically diagnosed with Kikuchi-Fujimoto disease and successfully treated with prednisone tapered over 3 months. She remains in clinical remission.


2020 ◽  
Vol 48 ◽  
Author(s):  
Lucimara Strugava ◽  
Ana Paula Rossa ◽  
Anny Raissa Carolini Gomes ◽  
Andressa Duarte Lorga ◽  
Betina Fabis Lautert ◽  
...  

Background: Megaesophagus is a chronic dilation of the esophagus rarely found in horses. It’s a non-specific disease that is associated with several causes, and esophageal hypomotility is the dysfunction that most commonly results in organ dilation. In the literature, there are few reports of megaesophagus in horses and, to date, no cases in mule have been reported. The objective of this work is to describe a case of a donkey with thoracic megaesophagus.Case: A 16 year-old donkey, castrated male, mixed breed weighing 195 kg, was referred for clinical care with a history of 5 days of anorexia. On physical examination, apathy, cachexia, 8% dehydration, moderate enophthalmos, ptialism, bilateral nasal discharge, dry and bristling hair were observed. Due to the poor general condition, a nasogastric tube was chosen to perform enteral nutrition, however, it was not possible to progress the tube to the stomach. In order to confirm the suspicion of a possible esophageal obstruction, gastroscopy was performed, where it was possible to observe an esophageal dilation filled with bulky food located in the thoracic portion of the esophagus. In an attempt to stimulate esophageal motility, in order to promote the progression of the material present in the region of dilation, intramuscular metoclopramide was administered (two applications every 6 h), however the treatment had no effect. Due to the unfavorable prognosis and financial limitations of the owner, euthanasia was performed, which was followed by autopsy and histopathological examination. At necropsy, a marked dilation of the esophagus was observed in the thoracic portion, which was filled with approximately 500 grams of bulky food (grass). In the mucosa of this area, multiple ulcers were observed that occasionally coalesce, covered by a moderate amount of friable and yellowish-white material.Discussion: Megaesophagus is characterized by organ dilatation and enlargement, absence of peristalsis, presence of tertiary contractions and non-total or partial relaxation of the lower sphincter. Its origin can be congenital or secondary (acquired). In the congenital form, which corresponds to the hypomotility and generalized dilation of the esophagus, the main consequence is the underdevelopment of the foal after weaning. The acquired form occurs due to motor changes in the esophagus or gastroesophageal sphincter, causing passive dilation of the organ. Lupus erythematosus, polymyositis, polyneuritis, degenerative neuropathies, hypoadrenocorticism, hypothyroidism, thiamine deficiency, heavy metal poisoning (Lead and Thallium), tumors (mainly thymoma) and cervical injuries are among the main causes of secondary megaesophagus. Animals with esophageal paralysis or some type of esophageal obstruction, usually present nasal and oral reflux of saliva and may develop aspiration pneumonia, accompanied by nutritional deficiencies, weight loss, changes in normal development and the presence of esophageal ulcers. With the exception of pneumonia, the animal in the present report had all these signs. The prognosis of animals affected by megaesophagus is unfavorable. One of the ways to alleviate this disease is surgical treatment, however, complications such as dehiscence are common after esophageal surgery, mainly due to the fact that this organ does not have a serous layer, resulting in delayed healing. For this reason, euthanasia is considered in most cases. Megaesophagus is a non-specific and multifactorial disease that affects horses, and there are no reports in the literature in mules. In this case, endoscopy allowed the diagnosis to be made while still alive, however it was not possible to determine the primary cause of the disease.


2018 ◽  
Author(s):  
Galith Kalmi

Introduction: Kikuchi-Fujimoto’s disease (KFD) or histiocytic necrotizing lymphadenitis is a benign and self-limited disease of unknown etiology mainly affecting young women. Although the association with systemic lupus erythematosus (SLE) is well described, no case of drug-induced lupus erythematosus (DILE) associated KFD has not been reported so far. Case report: We herein report a 25-year old Caucasian woman, with no medical history and no medication except for oral estrogen-progestin contraception (levonorgestrel-ethinylestradiol), who presented with cervical lymphadenopathy, fever and arthralgia without weight loss, night sweats or skin involvement. An exhaustive infectious disease screening was negative and lymph node biopsy revealed histiocytic necrotizing lymphadenitis suggesting KFD. Autoimmune screening tests evidenced high titers of anti-histone antibodies suggesting DILE induced by estrogen-progestin medication. The patient received a short course of non-steroidal anti-inflammatory treatment for painful lymphadenitis and arthralgia. Oral levonorgestrel-ethinylestradiol contraceptive medication was stopped and KFD and DILE completely recovered with a long-term disappearance of anti-histone antibodies. Conclusion: We report the first case of KFD associated-DILE following oral levonorgestrel-ethinylestradiol medication. Even though levonorgestrel-ethinylestradiol induced lupus is well known, the association with KFD has never been reported and the physiopathology remained unknown. Keywords: Kikuchi-Fujimoto disease, Lupus, Levonorgestrel-ethinylestradiol


2021 ◽  
Vol 12 (e) ◽  
pp. e33-e33
Author(s):  
Faten Rabhi ◽  
Refka Frioui ◽  
Nada Mansouri ◽  
Kahena Jabeur ◽  
Abderraouf Dhaoui

A 55-year-old male presented to our dermatology department with 3 months history of an asymptomatic and red raised plaque on the nose. There was no history of discoloration of skin prior to the development of this lesion. His only medical history was type 2 diabetes and high blood pressure. He had never undergone radiation therapy. On clinical examination, a well-defined glistening bluish-erythematous plaque 3 * 3 cm was present on the nose which was soft and non-tender (Fig. 1). Nasal mucosa was completely spared. No cervical lymphadenopathy was found. Head and neck examination was unremarkable. A CT scan of head and neck founded a soft tissue mass without infiltration of underlying structures. Figure 1: A well-defined glistening bluish-erythematous plaque 3*3 cm on the nose. DERMOSCOPY FINDINGS The dermoscopy revealed homogeneous pattern with combinations of colors (pinkish, bluish, and violaceous) and whitish veil areas without well-defined vessels or lacunae (Fig. 2). Figure 2: Homogeneous pattern with combinations of colors (pinkish, bluish, and violaceous) and whitish veil areas. HISTOLPATHOLOGY The histological examination of the first biopsy revealed a benign fibtoblastic reaction around normal vessels with fibrin deposits and without nuclear atypia. The second skin biopsy showed anastomosing vascular structures of variable diameter in the dermis, lined by plump endothelial cells with marked cellular, nuclear polymorphism and occasional mitosis, dissecting the collagen bundles (Fig. 3a). On immunohistochemistry, tumor cells expressed CD31, ERG, CD34 and VIII Factor. Ki67 was high in tumor cells (60%) (Fig. 3b). Figure 3: (a) HE x 400. Vascular proliferation with atypical endothelial cells (arrow). (b) IHC x 200, Elevated proliferation index Ki67.


1970 ◽  
Vol 24 (1) ◽  
pp. 19-28 ◽  
Author(s):  
MM Karim ◽  
SA Choudhury ◽  
MM Husain ◽  
MA Faiz

Findings of 80 patients of extra-pulmonary tuberculosis are described in this study. Most of the patients were under 30 years of age (71.2%), female patients were 56.3% and housewives were 37.3%. Lower socio-economic class were commonly affected (66.2%). Eighteen patients (22.5%) were smoker and almost equal number of cases had the history of intake of un-boiled milk. 44% patients were not vaccinated against tuberculosis. 36.2% patients had history of contact with tuberculous patients and 18.8% had previous history of tuberculosis. 70% patients had the history of fever and 30% had history of cough. Significant weight loss was noted in 85% patients. Lymph node tuberculosis was 36.2%, abdominal tuberculosis 35%. Cervical lymph nodes alone (37.9%) were commonly affected among the lymph node tuberculosis. Diagnosis was mainly based on histopathological examination or biopsy of specimen (97.5%) and demonstrations of AFB was possible in 2.5% cases. Along with surgical treatment medical treatment (chemotherapy) were prescribed in every patient. Forty-eight patients came for follow up. All responded to anti tubercular chemotherapy. (J Bangladesh Coll Phys Surg 2006; 24: 19-28)


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