Extranodal Rosai-Dorfman Disease With Mucosal Involvement of the Stomach in a Background of Autoimmune Atrophic Gastritis

2018 ◽  
Vol 26 (7) ◽  
pp. 671-675 ◽  
Author(s):  
Amanda Meindl ◽  
M. Sambasiva Rao ◽  
Guang-Yu Yang

Rosai-Dorfman disease (RDD), or sinus histiocytosis with massive lymphadenopathy, has been described involving both lymph nodes and extranodal sites, but extranodal RDD rarely involves the gastrointestinal tract. Although the etiology is unclear, several risk factors have been shown to be highly associated with this disease process, including viral infection and immune alterations. In this article, we present a case of a 79-year-old male with a history of autoimmune atrophic gastritis and multiple carcinoid tumors of the stomach presenting with a new stomach mass. An additional large sigmoid colon mass and adjacent enlarged lymph node was identified through imaging, prior to surgery. Through extensive pathologic analysis, we identified the first case of predominant extranodal RDD involving gastric mucosa and submucosa in a background of atrophic gastritis, with additional involvement of the sigmoid colon. Based on this case and literature review, we further discuss possible risk factors and pathogenesis of this disease process.

2017 ◽  
Vol 45 (2) ◽  
pp. 875-881 ◽  
Author(s):  
Ji Tu ◽  
Wen-Tian Li ◽  
Cao Yang

Rosai-Dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy is a rare benign disorder usually characterized by massive painless cervical lymphadenopathy and systemic manifestations. Extranodal involvement, especially spinal involvement, is extremely rare. We report a 41-year-old man who presented with only intermittent dorsodynia. His condition was diagnosed as non-specific inflammatory disease on the basis of preoperative puncture biopsy results. We performed total surgical resection. Histopathological findings showed distinctive emperipolesis and immunohistochemistry results were positive for cluster of differentiation CD68 and S100 and negative for CD1a. A good prognosis was confirmed at the 3-month follow-up visit. This is the first case of RDD of the subdural spine with such a long segment lesion. There is still no consensus regarding appropriate therapy for this type of RDD and the preoperative diagnosis remains challenging. The unusual presentation of our case serves as a reference when diagnosing and treating RDD.


2021 ◽  
Vol Volume 9 (upjohns/volume9/Issue2) ◽  
pp. 47-50
Author(s):  
N. Vishnu S. Reddy

ABSTRACT Rosai-Dorfman disease (RDD) is a rare histiocytic disorder commonly manifested as histiocytic proliferation of lymph nodes and extra nodal tissues initially described as a separate entity by Rosai and Dorfman under the term sinus histiocytosis with massive lymphadenopathy (SHML) in 1969. The treatment strategies can be variable and based on the severity of disease, v ital organ invol vement and pos s ible complications. The first case was a 47 years old female presenting with nasal obstruction and submandibular lymphadenopathy since few days. The second case was a 8 year old girl presented with stridor and lymphadenopathy in the submandibular region. In both the cases spontaneous resolution was expected and intervention was done only if there werecomplications. Patients were followed up every three months for two years and both the cases have resolved completely without any sequelae of the disease.Here we present two cases of Rosai-Dorfman disease presented to our hospital, this case series adds to the growing knowledge of Rosai-Dorfman di sease, along with under s tanding of pathophysiology, clinical diagnosis, treatment strategies and recovery options in cases where complications occurred.


2019 ◽  
Vol 12 (11) ◽  
pp. e231927
Author(s):  
Sweta Subhadarshani ◽  
Tarun Kumar ◽  
Sudheer Arava ◽  
Somesh Gupta

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a non-Langerhan cell histiocytosis which primarily involves lymph nodes. Extranodal involvement in the form of cutaneous plaques can occur and can pose a diagnostic challenge because of pleomorphic presentation and histopathological mimics. Rarely, systemic autoimmune involvement may complicate the disease process. We present a 28-year-old woman with slowly evolving scaly erythematous cutaneous plaques and fluctuating lymphadenopathy, associated with autoimmune haemolytic anaemia. The patient responded favourably to oral corticosteroids and acitretin with significant flattening of cutaneous plaques, reduction in size of neck nodes and improvement of anaemia.


2020 ◽  
Vol 06 ◽  
Author(s):  
Ravindra Verma ◽  
Vaibhav Misra ◽  
Dileep Tiwari ◽  
Prakash S. Bisen

Introduction: Many environmental risk factors are associated with some form of chronic inflammation. The spread of COVID-19 across the world has impacted every one of us. The first case of coronavirus was reported on 30 January 2020 in India originating from China. Study Area: India has a tremendous capacity to deal with the coronavirus outbreak because of its high immunity and climatic conditions. Maintaining social distancing and hand washing is not a sufficient step for preventing COVID-19. Indian system of traditional medicine has a potential worth to enhance immunity, which can resist a novel coronavirus. Material & Methods: A detailed study was carried out by analyzing national and international scientific databases (PubMed, SciFinder, ScienceDirect, Scopus, and Web of Science, Mendeley), thesis, and recognized books. Only Indian herbs with high immunity resistant power were analyzed. Epidemiologic studies with information on COVID-19 risk factors and precautions also considered for study purposes. Results: Some herbs like Ocimum tenuiflorum (Tulsi), Glycyrrhiza glabra (Liquorice), Curcuma domestica Vahl (Turmeric), Tinospora cordifolia (Giloy), Withania somnifera (Ashwagandha), Cinnamon (Dalchini), Shoot of Triticumaestivum Linn. (Wheatgrass), Andrographis paniculata (Kalmegh), can help in boosting immunity for COVID-19 disease. Discussion: Despite the shreds of evidence for the efficacy of these herbs in treating coronavirus induced infections; the proper dose with ideal timing for such interventions needs to verify in clinical trials. Researchers must have to take the privilege to explore the potential of herbs to reduce such epidemics of environmental threats.


Author(s):  
Carlo Lajolo ◽  
Rupe Cosimo ◽  
Schiavelli Anna ◽  
Gioco Gioele ◽  
Metafuni Elisabetta ◽  
...  

Background: Saprochaete clavata infection is an emerging issue in immunosuppressed patients, causing fulminant fungaemia. The purpose of this systematic review of cases is to retrieve all cases of S. clavata infection and describe oral lesions as the first manifestation of S. clavata infection. Methods: We report the first case of intraoral S. clavata infection in Acute Myeloid Leukemia (AML) affected subject, presenting as multiple grayish rapidly growing ulcerated swellings, and provide a review of all published cases of infection caused by S. clavata, according to PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines, conducted by searching SCOPUS, Medline, and CENTRAL databases. Only articles in English were considered. Individual patient data were analyzed to identify risk factors for S. clavata infection. Results: Seventeen of 68 retrieved articles were included in the review reporting data on 96 patients (mean age 51.8 years, 57 males and 38 females). Most cases were disseminated (86) with a 60.2% mortality rate. Ninety-five were hematological patients, with AML being the most common (57 cases). Conclusions:S. clavata infection in immunosuppressed patients has a poor prognosis: middle-age patients, male gender and Acute Myeloid Leukemia should be considered risk factors. In immunosuppressed patients, the clinical presentation can be particularly unusual, imposing difficult differential diagnosis, as in the reported case.


Author(s):  
Megan Culler Freeman ◽  
Stephanie Mitchell ◽  
John Ibrahim ◽  
John V Williams

Abstract Neonatal toxic shock syndrome (TSS)-like exanthematous disease (NTED) is a syndrome first reported in Japan. Neonates develop systemic exanthema, thrombocytopenia, and fever usually during the first week of life. The disease is distinguished from frank TSS because affected infants are not severely ill and do not meet TSS criteria. Most infants are confirmed to be colonized with TSST-1 producing strains of S. aureus. Suggested diagnostic criteria for NTED include a skin rash with generalized macular erythema and one of the following symptoms: fever >38.0°C, thrombocytopenia <150 x103uL, or low positive C-reactive protein (1-5 mg/dL) in the absence of another known disease process. NTED is common in Japanese NICUs, but outside Japan, only one case has been reported in France. We describe the first case of NTED reported in North America.


2011 ◽  
Vol 135 (12) ◽  
pp. 1597-1600 ◽  
Author(s):  
John J Cangelosi ◽  
Victor G Prieto ◽  
Doina Ivan

Rosai-Dorfman disease (RDD) is characterized histologically by a dense histiocytic infiltrate with emperipolesis and associated lymphocytes, plasma cells, and neutrophils. Eosinophils are not commonly associated. We report a patient with initial thymus and pituitary gland involvement by RDD, who later developed papules on the groin and axilla. Skin biopsies showed admixed histiocytic infiltrates (lymphocytes, neutrophils, and plasma cells) without emperipolesis. A prominent eosinophilic infiltrate was also observed, a feature not, to our knowledge, previously reported. Immunohistochemistry revealed positivity for CD68 (most cells) and S100 protein (scattered cells) and was negative for anti-CD1a. The diagnosis of RDD was established in the clinical context after comparison with the thymic and pituitary lesions (similar histologic features, albeit with fewer eosinophils, and immunohistochemical profiles). We present the first case, to our knowledge, of multicentric RDD with cutaneous involvement and associated prominent eosinophilic infiltrate. Thus, RDD should be included in the differential diagnosis of mononuclear infiltrates containing eosinophils.


2019 ◽  
Vol 153 (9) ◽  
pp. e52
Author(s):  
Marta Custódio ◽  
Isa Silva ◽  
Anabela Salgueiro Marques

2021 ◽  
Author(s):  
Ombretta Repetto ◽  
Valli De Re ◽  
Paolo Giuffrida ◽  
Marco Vincenzo Lenti ◽  
Raffaella Magris ◽  
...  

Abstract Background Autoimmune atrophic gastritis (AAG) is a chronic disease that can progress to gastric cancer (GC). To better understand AAG pathology, this proteomics study investigated gastric proteins whose expression levels are altered in this disease and also in GC. Methods Using two-dimensional difference gel electrophoresis (2D-DIGE), we compared protein maps of gastric corpus biopsies from AAG patients and controls. Differentially abundant spots (|fold change|≥ 1.5, P < 0.01) were selected and identified by LC–MS/MS. The spots were further assessed in gastric antrum biopsies from AAG patients (without and with Helicobacter pylori infection) and from GC patients and unaffected first-degree relatives of GC patients. Results 2D-DIGE identified 67 differentially abundant spots, with 28 more and 39 less abundant in AAG-corpus than controls. LC–MS/MS identified these as 53 distinct proteins. The most significant (adjusted P < 0.01) biological process associated with the less abundant proteins was “tricarboxylic acid cycle”. Of the 67 spots, 57 were similarly differentially abundant in AAG-antrum biopsies irrespective of H. pylori infection status. The differential abundance was also observed in GC biopsies for 14 of 28 more abundant and 35 of 39 less abundant spots, and in normal gastric biopsies of relatives of GC patients for 6 and 25 spots, respectively. Immunoblotting confirmed the different expression levels of two more abundant proteins (PDIA3, GSTP gene products) and four less abundant proteins (ATP5F1A, PGA3, SDHB, PGC). Conclusion This study identified a proteomics signature of AAG. Many differential proteins were shared by GC and may be involved in the progression of AAG to GC.


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