Traits as well as Treatments for Autoimmune DiseaseAssociated Cerebral Venous Nasal Thrombosis

Ollier disease is a rare nonhereditary skeletal disorder, characterized by multiple enchondromas, which are noncancerous growth of cartilage. In this report, we present a case of Ollier disease in a 10-year-old Syrian boy. The patient presented with multiple boney masses on hands; he had a history of pathological fractures when he was 5, which caused crippling. We analyzed the clinical, radiographical and pathological characteristics of our patient, which helped us to reach the final diagnosis. Ollier disease is a benign bone tumor, but it has a risk of malignant transformation into chondrosarcoma. The aim of this report is to document the presence of Ollier disease in Syria to help other Syrian physicians considering this disease in the differential diagnosis if they face similar presentations. © The Author(s) 2020. Published by Oxford University Press.Dermal sarcomas represent a group or rare malignancies of mesenchymal origin. Although surgical excision with wide margins can be curative, in the advanced/metastatic setting, treatment options are limited and the benefit from anthracycline-based chemotherapy or targeted agents is usually short-lived. Tumor mutational burden and PD-L1 expression scores can be used as predictive biomarker for response to immunotherapy in some metastatic cancers. The role of immune-checkpoint blockade for sarcoma patients remains investigational. Here we present three cases of dermal sarcomas with high TMB and PD-L1 expression and responses to anti-PD1 agents in two of them. © The Author(s) 2020. Published by Oxford University Press.Autoantibodies to leucine-rich glioma-inactivated protein 1 (LGI-1) are associated with inflammation of the limbic system. Faciobrachial dystonic seizures are pathognomonic for LGI1-antibiodies and their treatment with immunotherapy is effective in seizure control with a potential to prevent cognitive decline. We report a 57-year-old man who presented to the emergency department with recurrent seizures, visual hallucinations and severe memory impairment over a seven-week period; he reported a background of alcohol excess. Initial investigations revealed hyponatremia, indicating syndrome of inappropriate anti-diuretic hormone secretion. Magnetic resonance imaging of the brain revealed bilateral asymmetrical high-T2 and low-T1 signal in the medial temporal lobes. Serum immunofluorescence assay tested positive for LGI-1 antibody. Patient responded to treatment with levetiracetam, intravenous methylprednisolone and five plasma exchange sessions. Patient remains on a maintenance dose of prednisolone and azathioprine. It is imperative that clinicians recognize signs of autoimmune encephalitis in order to curb long-term sequelae and improve clinical outcomes. © The Author(s) 2020. Published by Oxford University Press.Plaque psoriasis is a chronic debilitating condition, and biologic agents that inhibit tumor necrosis factor-α (TNF-α) are widely employed in management of the condition. selleckchem Notwithstanding, several paradoxical adverse reactions have been reported with TNF-α inhibitors, including vasculitis, vitiligo, alopecia areata, sarcoidosis and other granulomatous diseases. Herein, we report the case of a 63-year-old man who developed vitiligo while on therapy with adalimumab following failure of conventional agents for plaque psoriasis. After discontinuation of adalimumab and initiation of secukinumab, vitiligo and other psoriatic symptoms gradually resolved. After 1 year of treatment, only small plaque areas were present in the flexor site with complete remission in the extensor area along with near complete resolution of depigmented areas. In this case of possible adalimumab-induced vitiligo in a patient with plaque psoriasis, secukinumab resolved both the symptoms of psoriasis and the likely adalimumab-related vitiligo. © The Author(s) 2020. Published by Oxford University Press.Fifty-five-year-old female with a past medical history of gastroesophageal reflux disease was admitted to hospital due to increased confusion, and muscle cramps for last 15 days. She was taking famotidine 20 mg twice a day for the last 2 years. She was alert and oriented to person and place only. She had dry skin, positive Chvostek's and Trousseau's sign. Blood work showed 141 mmol/L of sodium, 0.7 mg/dl of creatinine, 5.7 mg/dl of calcium, 0.55 mg/dl of magnesium, low PTH but normal parathyroid related peptide PTHrP, vitamin D (25) and vitamin D (1.25). She was discharged home on electrolyte supplements. She was readmitted with very low calcium and magnesium. Extensive workup including 24 h of urine calcium and magnesium was unimpressive. She was treated with IV therapy and discharged to follow up with nephrology in the clinic, and famotidine was discontinued on second discharge. Her calcium and magnesium levels remained normal, and in a few weeks later, oral electrolyte supplements were discontinued. © The Author(s) 2020. Published by Oxford University Press.Behcet's disease is a recurrent systemic vasculitic disorder. It manifests most commonly in the form of skin lesions, oral and genital ulcers and uveitis. Graves' thyrotoxicosis is an autoimmune disorder characterized by excessive production of thyroid hormones. We present a case of a 41-year-old male of Turkish descent who had symptoms of arthralgia, rash, palpitations and weight loss. Bloods tests showed raised inflammatory markers and biochemical evidence of severe autoimmune thyrotoxicosis. The patient was HLA-B51-negative, and pathergy test was inconclusive. A diagnosis of Behcet's disease was made on constellation of clinical symptoms. The patient was treated with carbimazole and prednisolone followed by azathioprine. The coexistence of Behcet's disease and Graves' disease in the same patient is very rare. Further studies are required to determine if there is a pathological association between these two conditions. © The Author(s) 2020. Published by Oxford University Press.Leptospirosis is a common disease between humans and animals characterized by a wide range of clinical manifestations. Erythema nodosum (EN) is a common clinical form of panniculitis or subcutaneous adipose inflammation caused by hypersensitivity responses to antigens, but the presence of EN in a subject with leptospirosis is a very rare case presentation. We will present a 42-year-old man with a headache, myalgia, nausea and rigid tender on the shin and both forearms, which was a typical form of EN lesions. © The Author(s) 2020. Published by Oxford University Press.