scholarly journals Varied presentations of cutaneous vasculitis: a case series

2020 ◽  
Vol 8 (8) ◽  
pp. 3066
Author(s):  
Pinky Gupta ◽  
Shweta Ganorkar ◽  
Surekha Bhalekar ◽  
Rajiv Rao
Keyword(s):  
Blood Vessels ◽  
Histopathological Examination ◽  
Wide Spectrum ◽  
Systemic Disease ◽  
Case Series ◽  
Clinical Severity ◽  
Cutaneous Vasculitis ◽  
Venous Stasis ◽  
Duration Of Symptoms ◽  
Organ Systems

Vasculitis involves a wide spectrum of clinicopathological process with reactive damage to the involved blood vessels. There is loss of vessel integrity instigating haemorrhage & luminal compromise leading to ischemia and necrosis of the tissue supplied by the involved vessels. It may affect varied size and type of blood vessels at different locations. It may be primary or secondary to systemic disease. It may involve a single organ like skin or may involve different organ systems at the same time. This case series include six cases of cutaneous vasculitis affecting different organs with varied presentations. Skin biopsies of six patients with unusual presentations were studied. Their complete history, physical examinations, laboratory investigations including serology were analysed and correlated with histopathological findings. The patients presented with different duration of symptoms varying from as short as 15 days to 1 year. Skin lesions were present in all cases while cardiac manifestation was seen in one. Serology and autoimmune disease markers were negative in all cases except one. However, histopathological features were in concordance with the clinical diagnosis of vasculitis. They were further classified as vasculitis secondary to Churg Strauss syndrome, venous stasis, Henoch Schonlein purpura or leucocytoclastic vasculitis.Vasculitis though a rare disease may manifest as an acute or chronic condition. It needs timely diagnosis by histopathological examination to aid in further management. It is important to assess the clinical severity in primary and secondary vasculitis, as it determines morbidity and mortality.

scholarly journals Multisystem inflammatory syndrome associated with SARS-CoV-2 infection in 45 children: a first report from Iran

2020 ◽  
Vol 148 ◽  
Author(s):  
Setareh Mamishi ◽  
Zahra Movahedi ◽  
Mohsen Mohammadi ◽  
Vahid Ziaee ◽  
Mahmoud Khodabandeh ◽  
...  
Keyword(s):  
Wide Spectrum ◽  
Case Series ◽  
Signs And Symptoms ◽  
Case Definition ◽  
Laboratory Findings ◽  
Duration Of Symptoms ◽  
Presenting Symptoms ◽  
Hands And Feet ◽  
Inflammatory Syndrome ◽  
D Dimer

Abstract During the coronavirus disease 2019 (COVID-19) pandemic, a new phenomenon manifesting as a multisystem inflammatory syndrome in children (MIS-C) which has a similar clinical presentation to Kawasaki disease, toxic shock syndrome and severe sepsis has emerged. Although the number of MIS-C reports is increasing, rare reports in Asia is still available. To our knowledge, this study is the largest series of published MIS-C cases in Iran. We performed a retrospective study of all patients with case definition for MIS-C admitted to the three paediatric hospitals in Iran. All of these hospitals are located within the most active COVID-19 pandemic areas (Tehran, Qom and Mazandaran) in Iran. Demographic characteristics, clinical data, laboratory findings, imaging and echocardiographic findings, treatment and outcomes were collected. Between 7 March and 23 June 2020, 45 children were included in the study. The median age of children was 7 years (range between 10 months and 17 years). Common presenting symptoms include fever (91%), abdominal pain (58%), nausea/vomiting (51%), mucocutaneous rash (53%), conjunctivitis (51%) and hands and feet oedema (40%) with median duration of symptoms prior to presentation of 5 (interquartile range (IQR) 3, 7) days. Fifty-three percent of children showed lymphopaenia. Overall, the majority of cases at admission had markedly elevated inflammatory markers erythrocyte sedimentation rate (ESR) (95.5%) and C-reactive protein (CRP) (97%). Ferritin was abnormal in 11 out of 14 tested patients (73%), and it was highly elevated (>500 ng/ml) in 47% of cases. Median fibrinogen level was 210 (IQR 165, 291) mg/dl, D-dimer was 3909 (IQR 848, 4528) ng/ml and troponin was 0.6 (IQR 0.1, 26) ng/ml, respectively. Twenty out of 31 patients (64.5%) had hypoalbuminaemia. In addition, hyponatraemia was found in 64% of cases. Twenty-five patients (56%) presented with cardiac involvement and acute renal failure was observed in 13 cases (29%). Pleural, ascitic, ileitis and pericardial effusions were found in 18%, 11%, 4% and 2% of cases, respectively. In conclusion, this is a first large case series of hospitalised children who met criteria for MIS-C in Iran. There was a wide spectrum of presenting signs and symptoms; evidence of inflammation with abnormal values of CRP, ESR, D-dimer, ferritin and albumin; and multi-organ involvement.

Cutaneous vasculitis, connective tissue diseases, and urticaria

2010 ◽  
pp. 4626-4655
Author(s):  
Susan Burge ◽  
Graham S. Ogg
Keyword(s):  
Connective Tissue ◽  
Blood Vessels ◽  
Systemic Vasculitis ◽  
Vascular Inflammation ◽  
Systemic Disease ◽  
Connective Tissue Diseases ◽  
Vascular Occlusion ◽  
Clinical Findings ◽  
Cutaneous Vasculitis ◽  
Imaging Studies

Vasculitis (angiitis) denotes necrotizing inflammation of the blood vessels; occlusive vasculopathy implies vascular occlusion without significant vascular inflammation. A small-vessel cutaneous vasculitis is the most common vasculitis affecting the skin, and may be the first sign of a systemic vasculitis, but 50% of patients have no systemic disease. The clinical findings must be integrated with the results of serological, pathological, and imaging studies to reach a diagnosis....

scholarly journals A Case of IgG4-Related Kidney Disease Developing While on Steroid Treatment for Autoimmune IgG4 Pancreatitis

2021 ◽  
Vol 9 ◽  
pp. 232470962110265
Author(s):  
Jonathan Vincent M. Reyes ◽  
Dawn Maldonado ◽  
Aaron S. Stern ◽  
Maritza Brown
Keyword(s):  
Kidney Disease ◽  
Inflammatory Process ◽  
Systemic Disease ◽  
Multiple Organ ◽  
Steroid Treatment ◽  
Autoimmune Process ◽  
Immunoglobulin G4 ◽  
Organ Systems ◽  
The Relationship ◽  
Steroid Sparing

IgG4 (immunoglobulin G4)-related systemic disease is an autoimmune process affecting multiple organ systems. This inflammatory process can present as but not limited to pancreatitis, cholangitis, or unspecified kidney disease. In this case, our patient developed IgG4-related kidney disease while already on a prolonged steroid course for IgG4-related pancreatitis. The patient ultimately had renal recovery after starting a higher dose of prednisone, but also developed steroid-related complications. This case further highlights the relationship between IgG4 diseases now termed IgG4-related systemic disease. This case brings to light the need for further investigative research into ideal steroid dosing, as well as steroid-sparing agents for IgG4-related systemic disease.

scholarly journals Imaging Evaluation of Male Breast Masses with Histopathologic Correlation: A Case Series

2021 ◽  
Author(s):  
Tanvi P. Vaidya ◽  
Subhash K. Ramani
Keyword(s):  
Magnetic Resonance Imaging ◽  
Wide Spectrum ◽  
Case Series ◽  
Male Breast ◽  
Radiological Evaluation ◽  
Resonance Imaging ◽  
Imaging Evaluation ◽  
Breast Masses ◽  
Female Breast

AbstractThe male breast can be afflicted with a wide spectrum of benign and malignant masses, similar to the female breast. A systematic radiological evaluation using mammography, ultrasonography, and when appropriate, magnetic resonance imaging, could aid this differentiation and provide clues to the diagnosis. In this article, we present six cases of male breast masses with an emphasis on the role of imaging in characterization and diagnosis.

Bilateral ocular surface squamous neoplasia: A study of 25 patients and review of literature

2021 ◽  
pp. 112067212110071
Author(s):  
Vijitha S Vempuluru ◽  
Monalisha Pattnaik ◽  
Neha Ghose ◽  
Swathi Kaliki
Keyword(s):  
Risk Factors ◽  
Ocular Surface ◽  
Histopathological Examination ◽  
Topical Steroid ◽  
Case Series ◽  
Intraepithelial Neoplasia ◽  
Excisional Biopsy ◽  
Ocular Surface Squamous Neoplasia ◽  
Retrospective Case

Purpose: To describe the risk factors, clinical presentation, management, and outcomes of patients with bilateral ocular surface squamous neoplasia (OSSN). Methods: Retrospective case series. Results: Of the 25 patients with bilateral OSSN, the mean age at diagnosis of OSSN was 31 years (median, 24 years; range, 2–60 years). Risk factors for bilateral OSSN included xeroderma pigmentosum ( n = 15, 60%), human immunodeficiency virus infection ( n = 3, 12%), conjunctival xerosis ( n = 1, 4%), and topical steroid use ( n = 1, 4%). There were no identifiable ocular or systemic risk factors in 7 (28%) patients. Presentation was synchronous in 14 (56%) and metachronous in 11 (44%) patients. Tumor morphology was bilaterally similar in 12 (48%) patients. Histopathological examination ( n = 36) revealed conjunctival intraepithelial neoplasia (CIN) grade 1 in 4 (8%); grade 2 in 7 (14%); carcinoma in situ in 5 (10%), and invasive carcinoma in 20 (40%). Primary management of OSSN ( n = 49) included excisional biopsy ( n = 31, 62%), topical immunotherapy (IFN α2B) ( n = 11; 22%), topical Mitomycin C (MMC) ( n = 3, 6%), enucleation ( n = 1, 2%), orbital exenteration ( n = 2, 4%), and plaque brachytherapy (PBT) ( n = 1, 2%). One patient was lost to follow-up after detection of tumor in the second eye. Recurrent tumors were noted in 16 (32%) eyes and binocular globe salvage was achieved in 16 (64%) patients at a mean follow up of 41 months (median 30 months; range, 1–164 months). Conclusion: OSSN occurrence can be synchronous or metachronous. Meticulous examination of the fellow eye is important for an early diagnosis of OSSN.

scholarly journals Diagnostic and severity scores for Cockayne syndrome

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
M. A. Spitz ◽  
F. Severac ◽  
C. Obringer ◽  
S. Baer ◽  
N. Le May ◽  
...  
Keyword(s):  
Early Diagnosis ◽  
Severity Score ◽  
Wide Spectrum ◽  
Genetic Disorder ◽  
Continuous Distribution ◽  
Cockayne Syndrome ◽  
Clinical Severity ◽  
Diagnostic Score ◽  
Clinical Diagnostic ◽  
Severity Scores

Abstract Background Cockayne syndrome is a progressive multisystem genetic disorder linked to defective DNA repair and transcription. This rare condition encompasses a very wide spectrum of clinical severity levels ranging from severe prenatal onset to mild adult-onset subtypes. The rarity, complexity and variability of the disease make early diagnosis and severity assessment difficult. Based on similar approaches in other neurodegenerative disorders, we propose to validate diagnostic and severity scores for Cockayne syndrome. Methods Clinical, imaging and genetic data were retrospectively collected from 69 molecularly confirmed CS patients. A clinical diagnostic score and a clinical-radiological diagnostic score for CS were built using a multivariable logistic regression model with a stepwise variable selection procedure. A severity score for CS was designed on five items (head circumference, growth failure, neurosensorial signs, motor autonomy, communication skills) and validated by comparison with classical predefined severity subtypes of CS. Results Short stature, enophtalmos, hearing loss, cataracts, cutaneous photosensitivity, frequent dental caries, enamel hypoplasia, morphological abnormalities of the teeth, areflexia and spasticity were included in the clinical diagnostic score as being the most statistically relevant criteria. Appropriate weights and thresholds were assigned to obtain optimal sensitivity and specificity (95.7% and 86.4% respectively). The severity score was shown to be able to quantitatively differentiate classical predefined subtypes of CS and confirmed the continuous distribution of the clinical presentations in CS. Longitudinal follow-up of the severity score was able to reflect the natural course of the disease. Conclusion The diagnostic and severity scores for CS will facilitate early diagnosis and longitudinal evaluation of future therapeutic interventions. Prospective studies will be needed to confirm these findings.

Hyper-eosinophilic syndrome: An uncommon cause of chronic abdominal pain in an elderly male

2021 ◽  
pp. 004947552098776
Author(s):  
Dibya L Praharaj ◽  
Bipadabhanjan Mallick ◽  
Preetam Nath ◽  
Sarat C Panigrahi ◽  
Anil C Anand ◽  
...  
Keyword(s):  
Abdominal Pain ◽  
Wide Spectrum ◽  
Hypereosinophilic Syndrome ◽  
Connective Tissue Disorder ◽  
Sleep Apnoea ◽  
Chronic Abdominal Pain ◽  
Elderly Male ◽  
Essentially Normal ◽  
Organ Systems ◽  
Obstructive Sleep

Hypereosinophilia is defined as an absolute eosinophil count of ≥1.5 × 109/L, and its presence with involvement of at least one organ system defines the hypereosinophilic syndrome. It may occur with parasitic infestation, connective tissue disorder or rarely in clonal disorders such as eosinophilic leucaemia. Organ systems that may be involved include the cardiovascular, central nervous, respiratory and gastrointestinal systems. In the latter, a wide spectrum of clinical presentation may be seen from trivial, to debilitating or rarely fatal. We report an elderly male with a history of bronchial asthma, obstructive sleep apnoea and food allergy who presented with chronic abdominal pain and weight loss. Abdominal examination and routine evaluation were essentially normal other than a peripheral hyper-eosinophilia. We witnessed a brisk and lasting response to an elimination diet and corticosteroids.

scholarly journals ARTHROSCOPIC LATARJET TECHNIQUE COMBINED WITH ENDOBUTTONS: FUNCTIONAL OUTCOMES IN 26 CASES

2018 ◽  
Vol 26 (5) ◽  
pp. 328-331 ◽  
Author(s):  
Alexandre Tadeu do Nascimento ◽  
Gustavo Kogake Claudio ◽  
Pedro Bellei Rocha ◽  
Juan Pablo Zumárraga ◽  
Olavo Pires de Camargo
Keyword(s):  
Shoulder Instability ◽  
Functional Outcomes ◽  
Short Form ◽  
Case Series ◽  
Anterior Shoulder Instability ◽  
Latarjet Procedure ◽  
Glenohumeral Instability ◽  
Duration Of Symptoms ◽  
Arthroscopic Latarjet ◽  
Arthroscopic Latarjet Procedure

ABSTRACT Objective: The cause of anterior shoulder instability is not fully understood and surgical management remains controversial. The objective of this study was to evaluate the results of patients undergoing arthroscopic Latarjet procedure with endobuttons. Methods: A retrospective study of 26 patients undergoing arthroscopic Latarjet procedure with endobuttons to treat anterior shoulder instability. Patients with previous glenohumeral instability, failure of Bankart procedure or Instability Severity Index Score (ISIS) greater than or equal to 6, were included. Patients were assessed by: DASH, UCLA, Rowe, Visual Analog Scale (VAS) of pain and Short-Form 36 (SF36) scores. Correct position and consolidation of the graft were evaluated. Results: Mean age was 31.5 years (16 to 46). Preoperative duration of symptoms was 1.7 years (1 month to 10 years). Mean follow-up was 14.3 (6 to 24) months. Mean postoperative scores were: 10 points in DASH; 1.6 in VAS, where 23 (88%) patients experienced mild pain and 3 (12%) moderate pain; 89 in Rowe; 32 in UCLA and 78 in SF-36. Positioning of the graft was correct in 25 (96%) cases, and was consolidated in 23 (88%). We had two cases of graft fracture (7%) and postoperative migration (7%). Conclusion: Surgical treatment using arthroscopic Latarjet with endobuttons is safe and effective, producing good functional outcomes in patients. Level of Evidence IV, Case Series.

scholarly journals Data Set for the Reporting of Malignant Odontogenic Tumors: Explanations and Recommendations of the Guidelines From the International Collaboration on Cancer Reporting

2018 ◽  
Vol 143 (5) ◽  
pp. 587-592 ◽  
Author(s):  
Pieter J. Slootweg ◽  
Edward W. Odell ◽  
Daniel Baumhoer ◽  
Roman Carlos ◽  
Keith D. Hunter ◽  
...  
Keyword(s):  
International Collaboration ◽  
Case Series ◽  
Initial Step ◽  
Odontogenic Tumors ◽  
Data Set ◽  
Expert Opinions ◽  
Organ Systems ◽  
Standardized Reporting ◽  
Selection Of ◽  
Cancer Reporting

A data set has been developed for the reporting of excisional biopsies and resection specimens for malignant odontogenic tumors by members of an expert panel working on behalf of the International Collaboration on Cancer Reporting, an international organization established to unify and standardize reporting of cancers. Odontogenic tumors are rare, which limits evidence-based support for designing a scientifically sound data set for reporting them. Thus, the selection of reportable elements within the data set and considering them as either core or noncore is principally based on evidence from malignancies affecting other organ systems, limited case series, expert opinions, and/or anecdotal reports. Nevertheless, this data set serves as the initial step toward standardized reporting on malignant odontogenic tumors that should evolve over time as more evidence becomes available and functions as a prompt for further research to provide such evidence.

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