scholarly journals Isolated Intracranial Rosai-Dorfman Disease

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Md. Taufiq ◽  
Abul Khair ◽  
Ferdousy Begum ◽  
Shabnam Akhter ◽  
Md. Shamim Farooq ◽  
...  
Keyword(s):  
Case Report ◽  
Ct Scan ◽  
Clinical Improvement ◽  
Cervical Lymphadenopathy ◽  
Rare Condition ◽  
Unknown Etiology ◽  
Nodal Involvement ◽  
Rosai Dorfman Disease ◽  
Parasellar Region ◽  
Significant Clinical Improvement

Background. Rosai-Dorfman disease (RDD) is a benign histiocytic proliferative disorder of unknown etiology. This rare condition commonly causes massive cervical lymphadenopathy. Intracranial RDD without any nodal involvement is extremely rare.Case Report. A young Bangladeshi male complained of bilateral complete blindness with left sided deafness for about three years. There was no lymphadenopathy. MRI and CT scan of brain suggested an inflammatory/neoplastic (?meningioma) lesion located at left parasellar region which extended frontally to encircle both optic nerves and also to left prepontine area. Histopathologically the lesion was diagnosed as RDD. The patient was treated with steroid and significant clinical improvement observed.Conclusion. The prognosis of intracranial RDD is not poor. It can be treated with surgery with or without corticosteroids, chemotherapy, and so forth. But as the condition is extremely rare and often misdiagnosed, the clinician, radiologist, and histopathologist should have a suspicion in their mind about the possibility of RDD.

scholarly journals A unique combination of Rosai-Dorfman disease and mycosis fungoides: a case report

2018 ◽  
Vol 6 ◽  
pp. 2050313X1877219 ◽  
Author(s):  
Amanda J Shelley ◽  
Nordau Kanigsberg
Keyword(s):  
Case Report ◽  
T Cell ◽  
Mycosis Fungoides ◽  
Cell Lymphoma ◽  
Cervical Lymphadenopathy ◽  
B Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Unknown Etiology ◽  
Unique Combination ◽  
Rosai Dorfman Disease

Rosai-Dorfman disease (RDD) is a rare histiocytic condition of unknown etiology. Patients with RDD classically present with massive painless cervical lymphadenopathy. However, extra-nodal disease occurs in approximately 40% of cases, with the skin being among the most commonly involved sites. Patients with isolated extra-nodal involvement may present without adenopathy. Reports of RDD occurring in patients with Hodgkin’s lymphoma, and B-cell lymphoma have been published, but there has only been one previous report of RDD in a patient with a T-cell lymphoma. This case report documents a unique combination of RDD and mycosis fungoides (MF), a cutaneous T cell lymphoma. This report also highlights diagnostic challenges in RDD due to the rarity of the condition and its variable presentation.

scholarly journals Erythromelanosis Follicularis Faciei et Colli: A Case Report in a Caucasian Male and Brief Review of the Literature

2020 ◽  
Vol 12 (3) ◽  
pp. 231-235
Author(s):  
Carl Maximilian Thielmann ◽  
Wiebke Sondermann
Keyword(s):  
Case Report ◽  
Family History ◽  
Clinical Presentation ◽  
Rare Condition ◽  
Unknown Etiology ◽  
Review Of The Literature ◽  
Caucasian Male

Erythromelanosis follicularis faciei et colli, a rare condition of unknown etiology, was first described by Kitamura et al. from Japan in 1960. It is characterized by a triad consisting of well-demarcated erythema, hyperpigmentation, and follicular papules. We report the case of a 50-year-old Caucasian male, who had asymptomatic symmetrical facial lesions since the age of 42. His family history was unremarkable. Published erythromelanosis follicularis faciei et colli cases of the last 10 years are summarized in this report to demonstrate the variability and differences in the clinical presentation of this uncommon diagnosis.

Cytomorphology and Histology Correlation of Rosai-Dorfman Disease: A 15-Year Study from a Tertiary Referral Centre in South India

2016 ◽  
Vol 61 (1) ◽  
pp. 55-61 ◽  
Author(s):  
Abid Hussain ◽  
Ashwani Tandon ◽  
Aruna K. Prayaga ◽  
Tara Roshni Paul ◽  
Anukonda M.V.R. Narendra
Keyword(s):  
Hodgkin Lymphoma ◽  
Plasma Cells ◽  
Cervical Lymphadenopathy ◽  
Cost Effective ◽  
Needle Aspiration ◽  
Unknown Etiology ◽  
Tertiary Referral Centre ◽  
Reliable Technique ◽  
Male Predominance ◽  
Rosai Dorfman Disease

Objectives: Rosai-Dorfman disease (RDD) is an uncommon, benign histiocytic disorder of unknown etiology, typically presenting in young adulthood. We highlight the cytomorphology of RDD and correlate it with the histopathology. Study Design: All cases diagnosed as RDD on fine-needle aspiration cytology between January 2001 and June 2015 were included. Clinical details were obtained from medical records. The cytology smears were reviewed along with the histopathology and immunohistochemistry, wherever available. Results: The study included 10 cases ranging in age from 11 to 68 years (median 29). There was a male predominance with a male:female ratio of 1.5:1. The patients commonly presented with bilateral cervical lymphadenopathy. Extranodal involvement was seen in 2 cases in the nose and mandible, respectively. Of these 10 cases, 8 were later biopsied. The cytological features included numerous crescentic histiocytes, emperipolesis, reactive lymphocytes and plasma cells. A histological diagnosis of RDD was made in 7 out of 8 cases, and 1 was diagnosed as Hodgkin lymphoma. Conclusion: FNA represents an efficient, minimally invasive, cost-effective and reliable technique for the diagnosis of RDD and may obviate the need for further biopsy. However, the disease has close differential diagnoses, including Langerhans cell histiocytosis, granulomatous lesions, and Hodgkin lymphoma. Hence, it must be remembered that there can be pitfalls when the diagnosis is made by cytology alone.

scholarly journals The importance of histopathology in the diagnosis of isolated renal sarcoidosis: a case report

2018 ◽  
Vol 40 (3) ◽  
pp. 291-295
Author(s):  
João Onofre Trindade Filho ◽  
Kaline Daniele de Souza Amaro ◽  
Allana Desirée Teixeira de Oliveira ◽  
Cecília Neta Alves Pegado Gomes ◽  
Hermann Ferreira Costa ◽  
...  
Keyword(s):  
Case Report ◽  
Medical Records ◽  
Clinical Laboratory ◽  
Histopathological Examination ◽  
Laboratory Data ◽  
Rare Condition ◽  
Unknown Etiology ◽  
Renal Manifestation ◽  
Systemic Inflammatory Disease ◽  
Rule Out

ABSTRACT Introduction: Sarcoidosis is a systemic inflammatory disease of unknown etiology, characterized by the presence of non-caseating granulomas in several organs; renal impairment alone is a rare condition. When it affects the kidneys, the most prevalent manifestations are hypercalcemia and hypercalciuria. This paper aims to address the topic of renal sarcoidosis, by means of a case report, and reinstate the importance of histopathology in its diagnosis. Methods: The data came from an observational clinical study with a qualitative approach, through an interview with the renal sarcoidosis patient and data from her medical records. Case report: Patient D.M.S., 50 years old, Caucasian, presented with reddish eyes and body pains lasting for fifteen days as first manifestations of the disease. Upon kidney ultrasound scan, we found renal parenchymal nephropathy. Serial renal function and metabolic tests reported anemia and progressive urea and creatinine changes, as well as hypercalcemia and hypercalciuria, confirming acute kidney failure (AKF). A histopathological examination suggested the diagnosis, which was confirmed by clinical, laboratory and histopathological data. There was therapeutic resolution after steroid therapy. Discussion: The symptomatology of sarcoidosis is diverse and often non-specific. Renal manifestation, which usually occurs after organ involvement, is present in less than 5% of patients, and about 1% to 2% of these patients may develop AKF. Conclusions: The use of histopathology together with clinical and laboratory data to diagnose isolated renal sarcoidosis, rule out other etiologies and introduce early treatment is of paramount importance.

scholarly journals Tolosa-Hunt: Case Report

2021 ◽  
Author(s):  
Lília Tereza Diniz Nunes ◽  
Flávia S. Silva ◽  
Karyme G. Aota ◽  
Maria Beatriz Miranda S. B. de Assis ◽  
João Fellipe B. Bento ◽  
...  
Keyword(s):  
Case Report ◽  
Cavernous Sinus ◽  
Clinical Care ◽  
Cranial Nerves ◽  
Rare Condition ◽  
Unknown Etiology ◽  
Specific Diagnosis ◽  
Hunt Syndrome ◽  
Diagnosis By Exclusion ◽  
History Of

Context: Tolosa-Hunt Syndrome (STH) is a rare condition with unknown etiology, it affects both genders equally. It is manifested by inflammation of the cavernous sinus and involvement of some cranial nerves pairs. Case report: MSR, 39 years, male, diver in the mining zone, history of recurrent otitis with acute pain and gradual hearing loss that progressed. He was admitted to the General Hospital of Palmas with symptoms of retrorbital headache. After physical exams it was found an ophthalmoplegia with right amaurosis and ipsilateral pain. The neurological examination showed a right eye with loss of photomotor reflex and presence of consensual reflex and eyelid ptosis. After 38 days in hospital, a probable septic thrombosis of the cavernous sinus was found, antibiotic and corticosteroids therapy was initiated. The patient also reports significant improvement in headache and partially in vision, he is currently hospitalized with clinical care and antibiotic therapy, awaiting results of the image examination report. Conclusions: Painful ophthalmoplegia in most cases is not diagnosed as STH. The differential diagnosis for this pathology is most often through brain magnetic ressonance and the ICHD-3 beta diagnostic criteria, STH should be suspected, but it is still necessary to close the diagnosis by exclusion, due to the lack of a specific diagnosis.

scholarly journals Rosai-Dorfman disease as a rare cause of cervical lymphadenopathy – case report and literature review

2018 ◽  
Vol 43 (3) ◽  
pp. 341-345 ◽  
Author(s):  
Aleksandra Miękus ◽  
Joanna Stefanowicz ◽  
Grażyna Kobierska-Gulida ◽  
Elżbieta Adamkiewicz-Drożyńska
Keyword(s):  
Case Report ◽  
Literature Review ◽  
Cervical Lymphadenopathy ◽  
Rosai Dorfman Disease

scholarly journals Case Report: Rosai-Dorfman Disease Involving Sellar Region in a Pediatric Patient: A Case Report and Systematic Review of Literature

2020 ◽  
Vol 7 ◽  
Author(s):  
Yi Zhang ◽  
Jie Liu ◽  
Jianyu Zhu ◽  
Xiang Zhou ◽  
Kun Zhang ◽  
...  
Keyword(s):  
Systematic Review ◽  
Case Report ◽  
Pediatric Patient ◽  
Cervical Lymphadenopathy ◽  
Sellar Region ◽  
Rosai Dorfman Disease ◽  
The Individual ◽  
Mass Lesions ◽  
Endocrine Abnormality

Rosai-Dorfman disease (RDD) is an extremely rare histiocytic disorder characterized by cervical lymphadenopathy, while the involvement of sellar region is less observed. Here we report a pediatric patient who was initially suspected as sellar germinoma but later identified as RDD. We also conducted a systematic review about RDD involving sellar region. A total of only 14 cases were included and analyzed in our study in terms of clinical presentation, endocrine abnormality, radiological features, pathology, treatment, and follow up. The most common neurological manifestations of sellar RDD is diabetes insipidus and visual changes. Two typical kinds of MRI manifestations were presented in sellar RDD; one is like meningioma-like mass lesions, another showing infiltrative pattern that demonstrates hyperintense areas on T2WI. Currently, the treatment of RDD is tailored to the individual clinical circumstances. For sellar RDD, surgical treatment can be considered to completely remove or debulk the tumor.

scholarly journals Rosai-Dorfman disease presenting with extensive cutaneous manifestation - Case report

2013 ◽  
Vol 88 (2) ◽  
pp. 256-259 ◽  
Author(s):  
Paula Azevedo Borges Leal ◽  
Adrilena Lopes Adriano ◽  
Marcelle Parente Breckenfeld ◽  
Igor Santos Costa ◽  
Antônio Renê Diógenes de Sousa ◽  
...  
Keyword(s):  
Case Report ◽  
Lymph Nodes ◽  
Cervical Lymphadenopathy ◽  
Cutaneous Manifestation ◽  
Common Site ◽  
Atypical Case ◽  
Inguinal Lymph Nodes ◽  
Rosai Dorfman Disease ◽  
Polyclonal Gammopathy ◽  
Extranodal Manifestations

Rosai-Dorfman disease is a benign, self-limited, idiopathic proliferative histiocytic disorder. It was first described in 1969 by Rosai and Dorfman. In its typical form the disease is characterized by extensive cervical lymphadenopathy associated with fever, polyclonal gammopathy and leukocytosis with neutrophilia. The skin is the most common site affected. Extranodal manifestations have been reported in 43% of cases. In this study, we report an atypical case of Rosai-Dorfman disease in a female with massive cutaneous manifestation on the thigh, associated with a minimal lymphadenopathy limited to the regional inguinal lymph nodes.

Seizure as a conversion symptom, a case report

2016 ◽  
Vol 33 (S1) ◽  
pp. S396-S396
Author(s):  
N. Salgado ◽  
S. Benavente ◽  
B. Macias ◽  
J.M. Coll
Keyword(s):  
Case Report ◽  
Ct Scan ◽  
Clinical Improvement ◽  
Pharmacologic Treatment ◽  
Short Stay ◽  
Psychiatric Unit ◽  
Clinical Presentations ◽  
Competing Interest ◽  
Made In

IntroductionPatients with conversive disorder could show atypical clinical presentations with neurological symptoms that are not frequently seen currently.Case ReportA 21-year-old female who was diagnosed of conversive disorder was admitted into a short-stay psychiatric unit for two weeks to introduce treatment and receiving a diagnosis. She presented few seconds long seizures in members without bitting her tongue and keeping control of sphincters, always surrounded by relatives. A neurological study was made with CT scan and electroencephalography and no evidences of neurological abnormalities were found. Various treatments were used but seizures went worse. Venlafaxine (150 mg/day) was prescribed after hipothymc reactive symptoms were observed, which together with pshycotherapy achieved clinical improvement in the two months follow-up.DiscussionPatients with conversive disorder don’t respond appropriately to pharmacologic treatment. In order for patients to understand the situation it is important to keep themupdated in an empathic manner. It is important to exclude other causes.ConclusionsA detailed psychopathological exploration should be made in all conversive patients, to explore symptoms and comorbidities that could reveal new therapeutic treatment.Disclosure of interestThe authors have not supplied their declaration of competing interest.

scholarly journals Rosai-dorfman disease of the fibula: A case report

2019 ◽  
Vol 26 (2) ◽  
pp. 108-113
Author(s):  
Glen Purnomo ◽  
Jesus Adrian A Dueñas ◽  
Richard S Rotor ◽  
Rafael S Claudio
Keyword(s):  
Case Report ◽  
Lymph Nodes ◽  
Immunohistochemical Staining ◽  
Cervical Lymphadenopathy ◽  
Skeletal System ◽  
Rosai Dorfman Disease

Rosai–Dorfman disease (RDD) is usually characterized by painless bilateral cervical lymphadenopathy associated with fever and leukocytosis. Although the disease may occur outside lymphnodes, manifestation of skeletal system occurs in less than 8% of cases. In addition, presentation of this disease in a purely skeletal form without lymph nodes involvement is extremely uncommon. This case report describes a 17-year-old female with a pure skeletal presentation of RDD in the fibula. Trocar biopsy was performed, and immunohistochemical staining using S100 and CD68 was done to confirm the diagnosis.

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