scholarly journals A case of systemic lupus erythematosus with thrombocytosis diagnosed by lymph-node biopsy.

1991 ◽  
Vol 14 (1) ◽  
pp. 79-84
Author(s):  
Shigeru Hosaka ◽  
Akira Ishikawa ◽  
Jun Okada ◽  
Hirobumi Kondo ◽  
Sadao Kashiwazaki
2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Yu Zuo ◽  
Michelle Foshat ◽  
You-wen Qian ◽  
Brent Kelly ◽  
Brock Harper ◽  
...  

Kikuchi Fujimoto’s disease (KFD) is a rare, immune-mediated, self-limiting disorder with unique histopathological features. KFD is usually seen in young Asian females; however, cases have been reported throughout the world and in all ethnicities. It has been recognized that there is a rare association between Systemic Lupus Erythematosus (SLE) and KFD via sporadic case reports. The exact pathophysiological relationship between these two diseases is still unclear. We report a case of a young Asian female who presented with persistent fever and lymphadenopathy and was diagnosed with Kikuchi Fujimoto’s disease based on lymph node biopsy; although an SLE workup was done, she did not meet the American Rheumatology Association (ARA) diagnostic criteria for lupus, and the lymph node biopsy did not show features of SLE. She improved clinically with a short course of steroid therapy. Two months later, the patient presented with central facial rash and arthralgia. SLE workup was repeated, a skin biopsy was done, and the results at this time supported a diagnosis of SLE.


2021 ◽  
Vol 15 (1) ◽  
Author(s):  
K. P. Jayawickreme ◽  
S. Subasinghe ◽  
S. Weerasinghe ◽  
L. Perera ◽  
P. Dissanayaka

Abstract Background Systemic lupus erythematosus is a rare autoimmune disorder, with the prevalence in Asia ranging from 30 to 50/100,000. The diagnosis of systemic lupus erythematosus is made according to the 2019 European League Against Rheumatism/American College of Rheumatology classification criteria, and it does not contain lymphadenopathy as diagnostic criteria. However, lupus lymphadenopathy has an estimated prevalence of 5–7% at the onset of disease, and 12–15% at any stage of the disease. Case presentation A 19-year-old Sinhalese girl had neck nodules since the age of 5 years, which increased in size and became tender since 1 year. She had alopecia and joint stiffness for 6 months. She presented with a 5-day history of worsening joint pain, fever, and painful, enlarging cervical nodules. She had tender cervical lymphadenopathy, and a vasculitic rash on both lower limbs. She had pancytopenia, an erythrocyte sedimentation rate of 92, positive antinuclear antibody titer, and high anti-double-stranded deoxyribonucleic acid (DNA), with low C3 and C4 complements. She had a high reticulocyte count of 5%, with direct and indirect antiglobulin tests being positive, indicating autoimmune hemolytic anemia. Lymph node biopsy showed moderate reactive follicular hyperplasia, with scattered plasma cells and immunoblasts, with varying degree of coagulative necrosis, suggestive of lupus lymphadenopathy. On immunohistochemistry of the lymph node biopsy, Bcl2 was negative, excluding lymphoma. Contrast-enhanced computed tomography of abdomen and chest was normal with no hepatosplenomegaly or lymphadenopathy. Skin biopsy showed leukocytoclastic vasculitis. Later, with development of generalized edema, she was found to have impaired renal function, and renal biopsy showed lupus nephritis. She was started on hydroxychloroquine, prednisolone, and mycophenolate mofetil, and her symptoms improved and lymphadenopathy regressed. Conclusion In the case of cervical lymphadenopathy in a patient with systemic lupus erythematosus, the possibilities of lupus lymphadenopathy, Kikuchi–Fujimoto disease, and lymphoma should all be considered, after excluding secondary infection due to immunosuppression. Histology confirms the differentiation of these pathologies. It is important to differentiate the cause for lymphadenopathy in systemic lupus erythematosus as the outcome and treatment varies. Lupus lymphadenopathy is usually generalized, but isolated cervical lymphadenopathy could also rarely be the first presentation of systemic lupus erythematosus. Lupus lymphadenopathy can be the only presenting feature, and needs a high index in suspecting systemic lupus erythematosus, though it is not included in the diagnostic criteria.


2015 ◽  
Vol 32 (4) ◽  
pp. 231-234
Author(s):  
Mohammad Rafiqul Islam ◽  
Abul Hayat Manik ◽  
Jannat Jeeba ◽  
Mohammod Omar Kasru ◽  
Rakib Hasan Mohammed ◽  
...  

Kikuchi Fujimoto’s disease (KFD) is a rare, immunemediated, self-limiting disorder with unique histopathological features. KFD is usually seen in young Asian females; however, cases have been reported throughout the world and in all ethnicities. It has been recognized that there is a rare association between Systemic Lupus Erythematosus (SLE) and KFD via sporadic case reports. The exact pathophysiological relationship between these two diseases is still unclear. We report a case of a young Asian female who presented with persistent fever followed by development of lymphadenopathy and was diagnosed as Kikuchi Fujimoto’s disease based on lymph node biopsy. Although an SLE workup was done and she initially did not meet the American Rheumatology Association (ARA) diagnostic criteria for lupus.The lymph node biopsy did not show typical features of SLE. At last criteria of SLE became obvious with time and case was diagnosed as SLE.J Bangladesh Coll Phys Surg 2014; 32: 231-234


Reumatismo ◽  
2019 ◽  
Vol 71 (2) ◽  
pp. 105-107
Author(s):  
C.A. Mansoor ◽  
Z. Shemin

Extranodal involvement in Kikuchi’s disease is uncommon. A 31-year-old previously healthy Indian woman was admitted with high grade fever, multiple joint pain and skin rash for 3 weeks. She had negative anti-nuclear antibodies and had features of Kikuchi’s disease on lymph node biopsy. She also had multiple extranodal manifestations including erythematous maculopapular rash, symmetric polyarthritis and hepatosplenomegaly. Kikuchi’s disease with extranodal involvement can clinically mimic diseases like hematological malignancies, connective tissue disorders and certain infections. A lymph node biopsy plays a crucial role in making an accurate diagnosis by excluding other diseases. A discussion on the importance of differentiating Kikuchi’s disease from systemic lupus erythematosus is included.


2019 ◽  
Vol 7 ◽  
pp. 232470961986229 ◽  
Author(s):  
Srikanth Naramala ◽  
Sharmi Biswas ◽  
Sreedhar Adapa ◽  
Vijay Gayam ◽  
Venu Madhav Konala ◽  
...  

We are reporting a case of a 63-year-old Chinese female who presented to the rheumatology clinic with positive antinuclear antibody and unintentional weight loss along with lymphadenopathy. Further workup revealed eosinophilia, elevated anti–double stranded DNA, serum protein, and serum IgG4 (immunoglobulin G4). The patient was diagnosed with systemic lupus erythematosus. Due to the raised IgG4 level along with eosinophilia and diffuse lymphadenopathy, IgG4-related systemic disease was suspected. It was confirmed with IgG4 staining on lymph node biopsy. Our case is presenting the fact that systemic lupus erythematosus and IgG4-related disease can be present in the same patient with multiple overlapping features making accurate diagnosis challenging.


2017 ◽  
Vol 42 (10) ◽  
pp. 787-789 ◽  
Author(s):  
Antoine Girard ◽  
Jessica Ohnona ◽  
Jean-François Bernaudin ◽  
Françoise Montravers ◽  
Claude Bachmeyer

2020 ◽  
Vol 11 (1) ◽  
Author(s):  
Sami Alhoulaiby ◽  
Lina Okar ◽  
Haya Samaan ◽  
Hisham Qalaani

Abstract Introduction Marfan syndrome, G6PD deficiency, systemic lupus erythematosus (SLE), and Castleman disease are four distinctive, thoroughly investigated entities whose coincidence was never reported. However, occurrence in pairs was sporadically mentioned in literature. Case presentation We report a 15-year-old Caucasian G6PD deficient Marfan male patient, who presented with tonic–clonic seizures, fever, a hemolytic episode, and general symptoms. After the discovery of hepatosplenomegaly, malar rash, and painless lymphadenopathy, further testing diagnosed a multifocal Castleman disease of the hyaline vascular subtype and systemic lupus erythematosus with lupus nephritis that got 35 points on the 2019 EULAR/ACR criteria. G6PD deficiency, SLE & Castleman disease, and seizures were handled medically with eventual improvement in the patient’s condition. Discussion and conclusion It is extremely rare to discover the gathering of these four diseases in the same patient. Marfan syndrome and G6PD deficiency were proven by respective clinical and laboratory examinations. Castleman disease that tends to occur in older age groups was confirmed via pathological study of a lymph node biopsy, which was compatible with the HHV-8 negative type reported in Asian countries. SLE is part of the differential diagnosis for Castleman disease, yet the newest evidence strongly supports its presence as a distinct entity. However, no concrete proof is available to suggest a causative relationship between the four of them, rather than a coincidental occurrence.


2018 ◽  
Vol 142 (11) ◽  
pp. 1341-1346 ◽  
Author(s):  
Anamarija M. Perry ◽  
Sarah M. Choi

Kikuchi-Fujimoto disease (KFD) is a rare entity characterized by subacute necrotizing lymphadenopathy and frequently associated with fever. Young adults of Asian ancestry are most commonly affected, but it has been reported worldwide. Despite many studies in the literature, the cause of KFD remains uncertain. Histologically, KFD is characterized by paracortical lymph node expansion with patchy, well-circumscribed areas of necrosis showing abundant karyorrhectic nuclear debris and absence of neutrophils and eosinophils. Three evolving histologic patterns—proliferative, necrotizing, and xanthomatous—have been recognized. By immunohistochemistry, histiocytes in KFD are positive for myeloperoxidase. There is a marked predominance of T cells in the lesions (with mostly CD8-positive cells) with very few B cells. The differential diagnosis of KFD includes infectious lymphadenitis, autoimmune lymphadenopathy (primarily systemic lupus erythematosus), and lymphoma. Clinicians and pathologists are poorly familiar with this entity, which frequently causes significant diagnostic challenges.


1999 ◽  
Vol 35 (3) ◽  
pp. 220-228 ◽  
Author(s):  
C Clercx ◽  
K McEntee ◽  
S Gilbert ◽  
L Michiels ◽  
F Snaps ◽  
...  

A case of concurrent canine systemic lupus erythematosus (SLE) and generalized bacterial infection in a six-year-old female Beauceron is reported. The dog presented with purulent nasal and ocular discharges, skin lesions (including seborrhea, hyperkeratotic areas, and papules as well as ecchymoses around the eyes, on both sides of the pinnae, and on the vulva), generalized lymph node enlargement, a mitral murmur, and lameness. Later, facial swelling, a retrobulbar abscess, and a cough also developed. Occurrence of a generalized bacterial infection was established by culture of group-C, beta-hemolytic Streptococcus from the throat, the mouth, a biopsy site (popliteal lymph node area), the retrobulbar abscess, and the lung. The diagnosis of SLE was based on the clinical signs and particularly on the occurrence of antinuclear antibody (ANA) and antidoublestranded-desoxyribonucleic acid (ds-DNA) antibody. Interestingly, the latter type of antibodies were also detected in two young female puppies whelped by this dog. Salient histological findings included an extreme cell depletion of the lymph nodes and spleen and severe pneumonitis and peribronchiolitis. The results of this case indicate that a definite diagnosis of canine SLE can, at times, be made on the basis of the presence of serum ANA and ds-DNA antibodies.


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