Treatment of scleredema adultorum of Buschke with intravenous immunoglobulin and mycophenolate mofetil in a 14-year-old girl: a case report.

BACKGROUND: Scleredema adultorum of Buschke is a rare disease characterized by firm and non-pitting edema of the skin. The condition is rare with unknown etiology. Diagnosis is made on the basis of clinical findings and skin biopsy. CASE PRESENTATION: Here, we describe a 14-year-old Iranian girl presenting with non-pitting edema and woody thickening of the skin that progressed within a month. She was evaluated for possible underlying malignancy or connective tissue disorders, which were excluded by multiple laboratory workups. She underwent a skin biopsy which confirmed the diagnosis of scleredema, and she was successfully treated with intravenous immunoglobulin and mycophenolate mofetil. CONCLUSION: While scleredema adultorum of Buschke is a rare disease with no definite treatment, our effort through this report was to highlight the possible benefits of treatment by intravenous immunoglobulin and mycophenolate mofetil.

Granulomatosis with polyangiitis with salivary glands involvement: Presenting a case and describing its clinical, pathophysiological, and therapeutic aspects.

Granulomatosis with polyangiitis (GPA), a rare form of small vessel vasculitis, may be manifested by multisystem involvement misleading its definitive diagnosis. The involvement of salivary glands is a very rare characteristic of GPA. Herein, we described a case of GPA with submandibular salivary gland involvement followed by reviewing the literature on similar cases. The case was a 31-year-old man, a known case of seronegative peripheral arthritis that referred recently with bilateral enlargement of the parotid and submandibular glands. Pulmonary nodules were also evident in the patient's CT scan. Fine-needle aspiration under ultrasound guidance indicated the presence of degenerated squamoid cells, giant cells, and inflammatory cells with a priority of neutrophils in the submandibular gland, as well as the presence of a cyst containing fluid without the evidence of malignancy in the parotid gland. The positivity for the Anti-neutrophil Cytoplasmic Antibody (C-ANCA) marker was also revealed. The patient was treated with methotrexate, prednisolone, and rituximab which led to a gradual reduction in the size of the glands and the improvement of the patient's clinical symptoms within 1 month after the treatment. Enlargement of salivary glands in the context of inflammatory disorders can raise doubts about the existence of GPA, and therefore imaging evaluation and histopathological assessment with an ANCA test will be necessary to confirm or rule out it.

Bilateral acute anterior uveitis and optic nerve edema as a manifestation of coronavirus disease-2019 (COVID-19): A case report.

Key clinical message: Acute anterior uveitis and optic disk edema could be a manifestation of COVID-19 infection, and healthcare providers should be aware of this possible consequence for in-time diagnosis and treatment. Abstract: Since the beginning of the coronavirus disease-2019 (COVID-19) pandemic, a wide range of clinical manifestations have been associated with this novel infection. The objective of this study was to show that acute anterior uveitis and optic disk edema could be possible manifestations of COVID-19 infection. The patient was a nine-year-old girl presenting with prolonged fever, myalgia, cough, diarrhea, and skin rashes. She also reported blurred vision, Photophobia, and eye redness. PCR test for COVID-19 returned positive. Imaging investigations showed pleural and pericardial effusion, mediastinal lymphadenopathy, and heart valve regurgitation. She was diagnosed with MIS-C and treated with methylprednisolone and IVIG. Bilateral acute anterior uveitis and optic disk edema was detected by slit lamp and fundus examination. She was successfully treated and follow-up ophthalmologic examinations showed improvement.

Cutaneous vasculitis after COVID-19 vaccination in a 41-year-old male.

Leukocytoclastic vasculitis could be a possible adverse event of different SARS-CoV-2 vaccines. Clinicians and manufacturers should be aware of this adverse event for appropriate diagnosis and treatment.

Acute respiratory distress syndrome after spontaneous rupture of a large pulmonary hydatid cyst in a 17-year-old male: A case report.

Pulmonary hydatid cysts (PHC) and their complications are still a health concern in endemic countries. Here we described a 17-year-old male presented with a large PHC with a spontaneous rupture. He developed acute respiratory distress syndrome (ARDS) requiring mechanical ventilation. He was treated with albendazole, broad-spectrum antibiotics, and corticosteroids. The patient's general condition did not allow any attempt for surgical resection of the cyst. He was discharged in stable condition after one month and referred to a thoracic surgeon for resection of the cyst. As far as we know ARDS after hydatid cyst rupture was rarely reported, and through this case report we aimed to raise awareness of this possible life-threatening complication.

Pulmonary vein thrombosis associated with metastatic uterine leiomyosarcoma: a case report.

BACKGROUND: Pulmonary vein thrombosis (PVT) is rarely associated with malignancies. Leiomyosarcoma, a malignant tumor originating from smooth muscles, has never been reported as the etiology of PVT. CASE PRESENTATION: In this case report, we described a 43-year-old Kurdish woman with a known case of leiomyosarcoma who presented with hemoptysis, dyspnea, and pleuritic chest pain. Chest computed tomography (CT) angiography revealed a thrombus in the left infero-posterior pulmonary vein. She was successfully treated with unfractionated heparin administered intravenously followed by orally administered warfarin. At the end of the article, we describe and compare other reports of malignancy-related PVT. CONCLUSIONS: While malignancies are not a common cause of PVT, both primary lung tumors and metastatic cancers could be associated with PVT. Delay in diagnosis may lead to serious complications and even death. Therefore, clinicians should be aware of the possibility of the development of PVT in different malignancies for appropriate diagnosis and treatment.

Tracheal Small Cell Carcinoma in a 52-year-old Male: A Case Report.

Primary small cell carcinoma of the trachea is a rare tumor fitting in the spectrum of neuroendocrine tumors. Due to the rarity and unspecific symptoms, the tumor is frequently misdiagnosed with other chronic lung diseases, and diagnosis is delayed. Here, we described a 52-year-old male presenting with dyspnea and cough. He had been treated with bronchodilators for months for suspected asthma or bronchiolitis without improvement. He had central wheezing on the general examination. Chest CT scan was unremarkable except for soft tissue prominence in the thoracic trachea. A large exophytic tumor was observed on bronchoscopy. A biopsy specimen was taken and revealed small cell carcinoma of the trachea. The patient succumbed to illness a short time after the bronchoscopy and before receiving any treatment. Our effort through this case report was to raise awareness of this rare tumor since a delay in diagnosis could lead to serious complications and even death.

Cardiac angiosarcoma: a case report of a young female with pulmonary metastasis.

BACKGROUND: Angiosarcoma is a malignant rare tumor that originates from vascular endothelial cells that cover lymphatic or blood vessels. Cardiac angiosarcoma is the most prevalent sarcoma entail the heart. It has low incidence rate and poor prognosis. Our effort through this report was raising awareness of uncommon manifestations of this disease and showing the importance of appropriate diagnosis and treatment. CASE PRESENTATION: We present a case of cardiac angiosarcoma in a young female whose symptoms included dyspnea and hemoptysis with a history of pericardial effusion and a past history of cardiac surgery for suspected atrial Myxoma. She had history of several hospitalizations and relapse of symptoms a few months after each hospital discharge. CONCLUSIONS: The unspecific symptoms of cardiac angiosarcoma made it difficult to make in time diagnose and appropriate treatment. Awareness of unspecific presentations of cardiac angiosarcoma is necessary for proper diagnosis and treatment while delayed diagnosis may worsen the prognosis and even lead to death.

Frequency, characteristics and outcome of corona virus disease 2019 (COVID-19) infection in Iranian patients with rheumatic diseases.

Aim of the work: To investigate the frequency, clinical characteristics and outcome of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in rheumatic diseases patients. Patients and methods: One thousand patients with rheumatic diseases including systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), psoriatic arthritis (PsA), axial spondyloarthritis (SpA), systemic sclerosis (SSc), Sjögren's syndrome (SS), Behçets disease (BD), vasculitis, idiopathic inflammatory myositis (IIM), relapsing polychondritis, sarcoidosis and antiphospholipid syndrome (APS) were studied. The following data were collected: age, sex, disease diagnosis, rheumatic disease medication. Rheumatic diseases patients were divided into two groups of infected and non-infected patients with COVID-19 and collected data were compared. Results: The 1000 patients mean age was 43.4 ± 13 years and 84.1% were females. The main diagnosis was RA (37.1%), followed by SLE (23.8%), SpA (13.4%), SSc (12.4%), vasculitis, BD and rhupus in 2.4%, 2.3% and 2.2% respectively, SS and SSc in 0.7% each. Most patients were taking glucocorticoids (78.4%). A large majority of patients were taking at least one of the cDMARDs. 16.1% were taking biologic therapy. 221 rheumatic diseases patients with COVID-19 were identified. Of these, 38 patients (17.2%) were hospitalized and 9 patients (4.1%) died. No significant difference was observed for compared variables in patients with and without COVID-19 except for prednisolone >20 mg/d (0.64% vs 2.26%; p = 0.048). Conclusion: Most rheumatic diseases do not seem to be a risk factor for developing COVID-19 infection and despite immunosuppressive therapies, there is no poorer outcome. Only, patients using prednisolone >20 mg/d are at higher risk of developing COVID-19 infection.

Systemic lupus erythematosus after coronavirus disease-2019 (COVID-19) infection: Case-based review.

Background: Coronavirus disease-2019 (COVID-19) is a novel infectious disease, which presents with various clinical manifestations. There is growing evidence of an association between COVID-19 infection and autoimmune diseases. The aim of this case report was to demonstrate the association of COVID-19 infection and the development of systemic lupus erythematosus (SLE). Case presentation: A 38 year old Iranian woman presented with progressive icterus, pleuritic chest pain, palpitation, dyspnea, photosensitivity and arthralgia 18-days after COVID-19 symptoms proved by a positive polymerized chain reaction (PCR). The chest and abdomen computerized tomography (CT) scan showed pericardial and pleural effusion and enlarged liver and abdominal lymph nodes. Antinuclear antibody (ANA), anti-double stranded deoxyribonucleic acid (anti-ds DNA) antibody and perinuclear anti-neutrophil cytoplasmic antibody (P-ANCA) were positive. She was diagnosed as SLE and was successfully treated with prednisolone 30 mg daily, hydroxychloroquine 200 mg daily and azathioprine 150 mg daily and she remarkably improved. Repeated anti-ds DNA antibody was positive. Due to nausea and abdominal discomfort, azathioprine was discontinued and replaced with mycophenolate mofetil 1500 mg daily. In the article, similar cases were presented; the mean interval between COVID symptoms and SLE presentations was 24.86 days. Pulmonary and renal involvements were the most common presentations of SLE triggered by COVID-19. The most frequently reported autoantibody was ANA. Conclusion: It is necessary to be aware of the development of lupus disease in COVID-19 infected patients, because prompt diagnosis and treatment is very important to improve their outcome.

Microscopic polyangiitis associated with coronavirus disease-2019 (COVID-19) infection in an elderly male.

Background: Microscopic polyangiitis (MPA) is an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) that affects predominantly small- sized vessels. A causal relationship between viral infections and vasculitis has been postulated.Aim of the workTo present the concomitant association of coronavirus disease 2019 (COVID-19) infection with MPA in an elderly Iranian male. Case presentation: A 67 year old Iranian man with the history of COVID-19 infection and a positive polymerized chain reaction (PCR) test four weeks before admission to the neurology department with acute onset pain, numbness and progressive weakness in both hands grip, sudden left foot drop and paresthesia. Erythrocyte sedimentation rate (ESR) was 95 mm/hr, ferritin 912 ng/ml and C-reactive protein (CRP) positive, proteinuria 1.1 g/24 h and markedly elevated perinuclear (P-ANCA): 526 IU/ml. Diagnosis of MPA was held presenting with mononeuritis multiplex, glomerulonephritis followed by diffuse alveolar hemorrhage and infiltration of lymphocytes in muscle fibers and vessels wall in sural nerve biopsy. He was successfully treated by methylprednisolone (1 g/day for 3 days) followed by 1 mg/kg with gradual tapering along with cyclophosphamide (CYC) (2 mg/kg). Intravenous immunoglobulin (IVIG, 2 g/kg in four divided doses) started for the management of inflammatory mononeuritis multiplex with gradual improvement. During hospitalization, plasmapheresis was performed due to alveolar hemorrhage for 5 day. The patient returned home on day 32 and followed-up in the rheumatology clinic with improvement of muscle power and handgrip strength. Conclusion: Vasculitis is potentially one of COVID-19's presenting symptoms and prompt diagnosis and treatment is crucial in improving outcome of patients.