Blurry Vision, Hearing Loss, and Constant Headache

2018 ◽  
pp. 49-54
Author(s):  
Aaron E. Miller ◽  
Tracy M. DeAngelis ◽  
Michelle Fabian ◽  
Ilana Katz Sand
Keyword(s):  
Pulmonary Involvement ◽  
Giant Cells ◽  
Skin Lesions ◽  
Neurological Involvement ◽  
Unknown Etiology ◽  
Inflammatory Disorder ◽  
Initial Symptoms ◽  
Blurry Vision ◽  
Systemic Manifestations ◽  
Noncaseating Granulomas

Sarcoidosis is a multisystem inflammatory disorder of unknown etiology, characterized pathologically by the presence of noncaseating granulomas and multinucleated giant cells. For unknown reasons, the disorder tends to be more common among African Americans and Scandinavians. Among the most common systemic manifestations are pulmonary involvement, lymphadenopathy, and skin lesions. Nervous system involvement occurs in 5–10% of cases. Despite the rarity of neurological involvement, 50–70% of the time, the neurological symptoms are the initial symptoms. In most patients with neurosarcoid, there is evidence for involvement in other systems, either at presentation or during the course of the condition. The presentation in neurosarcoidosis is varied. Steroids are the first-line treatment, but some patients may also require steroid-sparing agents to control the condition.

scholarly journals Immunopathogenesis of Ocular Behçet’s Disease

2014 ◽  
Vol 2014 ◽  
pp. 1-13 ◽  
Author(s):  
Un Chul Park ◽  
Tae Wan Kim ◽  
Hyeong Gon Yu
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Retinal Vasculitis ◽  
Skin Lesions ◽  
Unknown Etiology ◽  
Ocular Involvement ◽  
Inflammatory Disorder ◽  
General Hypothesis ◽  
Behcet's Disease

Behçet’s disease (BD) is a chronic recurrent systemic inflammatory disorder of unknown etiology characterized by oral and genital ulcerations, skin lesions, and uveitis. The ocular involvement of BD, or Behçet’s uveitis (BU), is characterized by panuveitis or posterior uveitis with occlusive retinal vasculitis and tends to be more recurrent and sight threatening than other endogenous autoimmune uveitides, despite aggressive immunosuppression. Although pathogenesis of BD is unclear, researches have revealed that immunological aberrations may be the cornerstone of BD development. General hypothesis of BD pathogenesis is that inflammatory response is initiated by infectious agents or autoantigens in patients with predisposing genetic factors and perpetuated by both innate and acquired immunity. In addition, a network of immune mediators plays a substantial role in the inflammatory cascade. Recently, we found that the immunopathogenesis of BU is distinct from other autoimmune uveitides regarding intraocular effector cell profiles, maturation markers of dendritic cells, and the cytokine/chemokine environment. In addition, accumulating evidence indicates the involvement of Th17 cells in BD and BU. Recent studies on genetics and biologics therapies in refractory BU also support the immunological association with the pathogenesis of BU. In this review, we provide an overview of novel findings regarding the immunopathogenesis of BU.

scholarly journals Chronic Inflammatory Arthropathy Preceding Acute Systemic Manifestations of Sarcoidosis: A Possible Overlap of Idiopathic Juvenile Arthritis and Sarcoidosis

2019 ◽  
Vol 2019 ◽  
pp. 1-8 ◽  
Author(s):  
Matheus Campello Vieira ◽  
Priscilla Gomes Tosta ◽  
Fernanda Morello Nicole ◽  
Lucas Enock V. Roberto ◽  
Júlia Guasti P. Vianna ◽  
...  
Keyword(s):  
Elevated Serum ◽  
Lymphoproliferative Disease ◽  
Juvenile Arthritis ◽  
Unknown Etiology ◽  
Multisystem Disease ◽  
Idiopathic Juvenile Arthritis ◽  
Diagnostic Hypothesis ◽  
Liver Surface ◽  
Systemic Manifestations ◽  
Noncaseating Granulomas

Sarcoidosis is a multisystem disease with unknown etiology, marked by T lymphocytes and macrophages agglomeration, which leads to the formation of noncaseating granulomas in the affected tissues. We describe a case of a 40-year-old black patient referred to our service for evaluation of nephrolithiasis and persistent elevation of plasma creatinine. He reported important weight loss, fever episodes, and abdominal and low back intermittent pain in the past 6 months. The investigation revealed elevated serum calcium level, hepatosplenomegaly, retroperitoneal lymphadenopathy, anemia, thrombocytopenia, and nephrolithiasis. The initial diagnostic hypothesis was lymphoproliferative disease, but the laparoscopic propaedeutic showed multiple white lesions on the liver surface, which biopsy identified as noncaseating granulomas with asteroid corpuscles, suggestive of sarcoidosis. He was treated with corticosteroids with significant improvement in symptoms and in calcium and creatinine levels. Besides, the patient presented a long-term large joints arthropathy, especially on the knees (with bilateral prosthesis), wrists, and ankles, of unknown etiology. We discuss the systemic manifestations of sarcoidosis related to the reported case, as well as the possible overlapping of idiopathic juvenile arthritis with sarcoidosis.

scholarly journals Sarcoidosis and Its Splenic Wonder: A Rare Case of Isolated Splenic Sarcoidosis

2018 ◽  
Vol 2018 ◽  
pp. 1-4 ◽  
Author(s):  
Khushali Jhaveri ◽  
Abhay Vakil ◽  
Salim R. Surani
Keyword(s):  
Rare Case ◽  
Hematologic Malignancies ◽  
Pulmonary Involvement ◽  
Interesting Case ◽  
Organ System ◽  
Unknown Etiology ◽  
Granulomatous Disease ◽  
Uncommon Presentation ◽  
The Many ◽  
Noncaseating Granulomas

Sarcoidosis is a systemic granulomatous disease of unknown etiology characterized by presence of noncaseating granulomas in the involved organs. The pulmonary interstitium is most commonly affected but extrapulmonary involvement can occur in almost any other organ system. Such an involvement can occur with or without the presence of pulmonary involvement, but isolated extrapulmonary involvement has been noted only in around 10% of cases. Isolated splenomegaly is very rare and an uncommon presentation of sarcoidosis. It is clinically challenging because of the extensive differential diagnosis. Among the many considerations are hematologic malignancies, primary splenic or metastatic tumors, infiltrative disorders, inflammatory disorders, and infections. We hereby discuss an interesting case of a 40-year-old female with isolated splenic sarcoidosis.

Neurosarcoidosis

2013 ◽  
Author(s):  
Aaron E. Miller ◽  
Teresa M. DeAngelis
Keyword(s):  
Nervous System ◽  
Giant Cells ◽  
Unknown Etiology ◽  
Multinucleated Giant Cells ◽  
Systemic Sarcoidosis ◽  
Granulomatous Disorder ◽  
Noncaseating Granulomas

Sarcoidosis is a multi-systemic, inflammatory, granulomatous disorder of unknown etiology, characterized pathologically by the presence of noncaseating granulomas and multinucleated giant cells. Involvement of the nervous system is estimated in 5% of patients with systemic sarcoidosis, and rarely occurs as an isolated phenomenon. In this chapter, we review the most common peripheral and central presentations of neurosarcoidosis.

Cutaneous Sarcoidosis

2020 ◽  
Vol 41 (05) ◽  
pp. 689-699
Author(s):  
Avrom Caplan ◽  
Misha Rosenbach ◽  
Sotonye Imadojemu
Keyword(s):  
Unknown Etiology ◽  
Inflammatory Disorder ◽  
Cutaneous Sarcoidosis ◽  
Calcinosis Cutis ◽  
Ongoing Research ◽  
Cutaneous Manifestations ◽  
Normal Organ ◽  
Disease Prognosis ◽  
Noncaseating Granulomas ◽  
The Relationship

AbstractSarcoidosis is a chronic, multisystem, inflammatory disorder of unknown etiology that is characterized by noncaseating granulomas that impair normal organ functioning. Sarcoidosis predominantly affects the lungs, but the skin is often cited as the second most frequently involved organ. Cutaneous manifestations of sarcoidosis are highly variable and ongoing research seeks to better understand the relationship between clinical morphology and disease prognosis. Skin findings in patients with sarcoidosis can be “specific,” in which sarcoidal granulomas infiltrate the skin, or they can represent a “nonspecific” reactive inflammatory process, as is seen in calcinosis cutis and erythema nodosum. Cutaneous sarcoidosis can be the initial presenting sign or develop later in the course of the disease. In some patients, the skin will be the most involved and impactful organ system and will drive therapy. In other cases, the skin will be an incidental or minor finding, but may be easily accessible for biopsy to confirm the diagnosis. There are many potential therapies for sarcoidosis, though no one therapy is universally effective.

scholarly journals Multicentric Reticulohistiocytosis Presenting with Papulonodular Skin Lesions and Arthritis Mutilans

2013 ◽  
Vol 2013 ◽  
pp. 1-4
Author(s):  
Raya Saba ◽  
Shawn G. Kwatra ◽  
Bishwas Upadhyay ◽  
Aibek E. Mirrakhimov ◽  
Farah N. Khan
Keyword(s):  
Histopathological Examination ◽  
Giant Cells ◽  
Skin Lesions ◽  
Left Knee ◽  
Unknown Etiology ◽  
Multisystem Disorder ◽  
Internal Organs ◽  
Multicentric Reticulohistiocytosis ◽  
Eosinophilic Cytoplasm ◽  
Mucous Membranes

Multicentric reticulohistiocytosis is a rare multisystem disorder of unknown etiology that is characterized by erosive polyarthritis and papulonodular lesions on the skin, mucous membranes, and internal organs. We report the case of a 54-year-old female who was misdiagnosed as having rheumatoid arthritis and underwent numerous joint replacement surgeries for progressively destructive arthritis in her hands, shoulders, hips, and knees. The patient finally received a diagnosis of multicentric reticulohistiocytosis after histopathological examination of the patient’s left knee arthroplasty which revealed a diffuse histiocytic infiltrate, multinucleated giant cells, and finely granulated eosinophilic cytoplasm with a ground-glass appearance.

scholarly journals An Uncommon Cause of a Small-Bowel Obstruction

2017 ◽  
Vol 2017 ◽  
pp. 1-4
Author(s):  
Ali Zakaria ◽  
Bayan Al Share ◽  
Issam Turk ◽  
Samira Ahsan ◽  
Waseem Farra
Keyword(s):  
Small Bowel ◽  
Small Bowel Obstruction ◽  
Bowel Obstruction ◽  
Previous History ◽  
Granulomatous Inflammation ◽  
Giant Cells ◽  
Unknown Etiology ◽  
Granulomatous Disease ◽  
History Of ◽  
Noncaseating Granulomas

Sarcoidosis is a systemic granulomatous disease of unknown etiology, characterized by the formation of noncaseating granulomas. Gastrointestinal (GI) system involvement that is clinically recognizable occurs in less than 0.9% of patients with sarcoidosis, with data revealing small intestine involvement in 0.03% of the cases. A high index of suspension is required in patients presenting with small-bowel obstruction and previous history of sarcoidosis. Establishing a definitive diagnosis of GI sarcoidosis depends on biopsy evidence of noncaseating granulomas, exclusion of other causes of granulomatous disease, and evidence of sarcoidosis in at least one other organ system. Treatment of GI sarcoidosis depends on symptomatology and disease activity. Herein, we are presenting a case of 67-year-old female patient who had acute small-bowel obstruction at the level of jejunum with postoperative histopathologic evidence of noncaseating granulomatous inflammation with multinucleated giant cells, consistent with sarcoidosis.

scholarly journals THU0590 WHEN RARE IS EVEN RARER: A COMPLEX CASE OF BEHCET DISEASE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 537.3-537
Author(s):  
M. DI Cicco ◽  
O. M. Epis ◽  
C. Casu ◽  
A. Adinolfi ◽  
L. Alvaro ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Vascular Complications ◽  
Artery Aneurysm ◽  
Skin Lesions ◽  
Complex Case ◽  
Behçet Disease ◽  
Inflammatory Disorder ◽  
X Rays ◽  
Behcet Disease ◽  
Intracardiac Thrombosis

Background:Behcet disease is a rare inflammatory disorder with the unique ability to affect vessels of any size. The disease could be associated to thrombosis in both the venous and arterial compartment, and often aneurysms. In particular, the presence of aneurysms of the pulmonary artery is rarely, if ever, seen in conditions other than Behcet. Cardiac involvement, albeit uncommon, is also described and associated to a severe prognosis. The treatment is based on immunosuppressants, meanwhile the use of anticoagulants -especially when aneurysms are present- is debated.Objectives:To describe a complex case of Behcet disease.Methods:We report the case of a 45 years old man of Chinese origin who presented to A&E with fever and acute dyspnea. Blood test revealed raised ESR and CRP and raised neutrophil count. Chest X rays showed bilateral opacities suggesting pneumonia. The patient did not improve over the course of antibiotics. Later on, he presented with an episode of hemoptysis and worsening dyspnea, so he was admitted to the Intensive Care Unit. CT showed bilateral pulmonary thromboembolism and aneurysm of the pulmonary artery. Echocardiogram and cardio-MRI revealed a large, mobile thrombus within the right atrium. Extensive work-up for infections and cancer was unrevealing. ANA, ENA and ANCA antibodies were negative. On the basis of a past medical history of recurrent oral ulcers and papulopustular skin lesions that patient admitted on questioning, a diagnosis of Behcet disease was suspected. In keeping with that, HLA-B51 turned out positive. The patient was promptly started on IV steroid pulses followed by Cyclophosphamide 1 gr IV monthly for six months, then on IV anti-TNF alpha Infliximab. He was also commenced on low molecular weight heparin (LMWH) and subsequently direct factor Xa inhibitor Apixaban.Results:The patient improved significantly with progressive regression of the pulmonary CT changes. He was discharged and able to get back to his daily life activities. After 2 years and a half of treatment, the aneurysm was stable and the intracardiac thrombus completely cleared.Conclusion:This case is of particular interest because of the concomitant presence of two rare vascular complications of Behcet disease-intracardiac thrombosis (<1-2%, less than 100 cases described worldwide) and pulmonary artery aneurysm (1-2%). Prompt introduction of immunosuppressant therapy was associated with a favorable outcome with no recurrence. We could speculate that, to some extent, the concomitant use of anticoagulants may have contributed to the complete resolution of the intracardiac thrombosis.Disclosure of Interests :MARIA DI CICCO: None declared, oscar massimiliano epis Consultant of: yes, Speakers bureau: yes, Cinzia Casu: None declared, Antonella Adinolfi: None declared, Luisa Alvaro: None declared, Valeria Campanella: None declared, Michel Chevallard: None declared, Marina Muscarà: None declared, Mariaeva Romano: None declared, Emanuela Schito: None declared, Nicola Ughi: None declared, Elisa Verduci: None declared, Davide Antonio Filippini: None declared

P01-228-Behcet's syndrome comorbid with bipolar disorder: A case report

2011 ◽  
Vol 26 (S2) ◽  
pp. 229-229
Author(s):  
F. Maner ◽  
Ö. Şahmelikoğlu ◽  
Ö. Hısım ◽  
H. Özhan ◽  
H. Sarıahmetoğlu ◽  
...  
Keyword(s):  
Bipolar Disorder ◽  
Manic Episode ◽  
Unknown Etiology ◽  
Inflammatory Disorder ◽  
Behçet’S Syndrome ◽  
Psychomotor Activity ◽  
Chronic Inflammatory Disorder ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

IntroductionBehcet's Syndrome is a chronic inflammatory disorder of unknown etiology, characterized by aphthous lesions and recurrent ulceration of the mouth, genitals and uveitis.ObjectivesThe central nervous system is involved in about 20% of cases.AimsOnly few reports deal with affective symptoms associated with Behcet's syndrome.MethodsWe report a case of a 43 year old male with Neuro-Behcet's Syndrome that presents with a psychotic manic attack. He developed Behcet's Syndrome at the age of 23, with recurrent uveitis and aphthous lesions in the mouth, painful ulcers in the genitalia and erythema nodosum. HLA-B 5 was positive.ResultsHe was treated with azothioprine 150 mg/day for 13 years and prednole 100 mg/day during uveitis attacts for a week. At the age of 37 a sudden occurrence of right hemiparesia due to cerebrovascular accidence salicylic acid 100mg/day, siclosporine 150 mg/day, piracetame 1600mg/day were administered. He presented to psychiatry clinic in manic episode with euphoric mood, psychomotor agitation, talkativeness, decreased need for sleep, excessive buying and he had an unrealistical thought that he was a player of a famous soccer team. He was diagnosed as bipolar I disorder, according to DSM-IV. This was the patient's first admission and the symptoms which were continuing for 6 years exaggerated during uveitis attacks.Psychiatric examination releaved that increaced psychomotor activity, hypomaniac affect, amount and affect speed of speech affect, increased associations, grandiose delusions.ConclusionThere are a few reports dealing with bipolar disorder as an entity related to Behcet's syndrome.

NONTUBERCULOUS ACID-FAST BACILLI IN CERVICAL ADENITIS

PEDIATRICS ◽  
1957 ◽  
Vol 20 (4) ◽  
pp. 688-697
Author(s):  
Haddow M. Keith ◽  
Lyle A. Weed ◽  
Gerald M. Needham
Keyword(s):  
Giant Cells ◽  
Skin Lesions ◽  
Mycobacterium Ulcerans ◽  
Cervical Region ◽  
Tubercle Bacillus ◽  
Cervical Lymph Nodes ◽  
Caseous Necrosis ◽  
Acid Fast Bacilli ◽  
Cervical Adenitis ◽  
Bona Fide

THE TUBERCLE bacillus is the most common cause of lymphadenitis with caseous necrosis. Such a condition in the cervical region, while less frequent than in previous decades, still occurs occasionally. While other agents, for example, Coccidioides, Histoplasma, Brucella, Pasteurella and Miyagawanella, are known to produce a similar histopathologic reaction, they are not commonly associated with lesions in the cervical lymph nodes, especially in children. Therefore the finding of acid-fast bacilli in such lesions has generally been considered bona fide evidence of tuberculosis, although previous reports from this clinic indicate that such is not necessarily true. It is recognized that there are other acid-fast bacilli, such as the lepra and smegma bacilli, which may or may not cause disease. In 1944 Gellerstedt reported seven cases of tuberculoid skin lesions due to atypical acid-fast bacilli. He considered these to be due to exogenous infection with acid-fast bacilli differing from the organisms found in tuberculosis, and he considered them as possibly saprophytic organisms. In 1948 MacCallum and co-workers reported six cases of skin lesions due to acid-fast organisms that were not Mycobacterium tuberculosis. The lesions did not contain tubercle follicles, giant cells or caseous material. The name "Mycobacterium ulcerans" was applied to these organisms by Australian workers. In 1954 Linnell and Nordén described skin lesions that occurred in 80 boys and girls who used a certain swimming pool in a Swedish town. The etiologic organisms were acid fast, differed from those described by MacCallum and associates and were not lepra bacilli. Guinea pigs injected with these organisms presented no evidence of lesions after 7 weeks.

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