scholarly journals Epidemiological and clinical characteristics of patients with pemphigus treated in Białystok in 2001-2018

2020 ◽  
pp. 457-465
Keyword(s):  
Rural Areas ◽  
Clinical Characteristics ◽  
Pemphigus Vulgaris ◽  
Skin Lesions ◽  
Immunosuppressive Agent ◽  
Pemphigus Foliaceus ◽  
Aim Analysis ◽  
North East ◽  
Oropharyngeal Cavity ◽  
Medical University

BACKGROUND. Pemphigus is a group of potentially fatal dermatological autoimmune disorders. AIM. Analysis of cases of pemphigus vulgaris (PV) and pemphigus foliaceus (PF) newly diagnosed and treated at the Department of Dermatology and Venereology, Medical University of Białystok, North-east Poland in years 2001-2018. MATERIAL AND METHODS. A retrospective analysis and comparison of sociodemographic, epidemiological and clinical characteristics of PV and PF patients, including: age, gender, residency, initial severity of skin lesions, involvement of mucous membranes, co-morbidities and their treatment, efficacy of therapy. RESULTS. Sixty-two new cases – 41 (66.13%) of PV and 21 (33.87%) of PF were diagnosed. The average age of PV patients was 54.85±12.35 years and those of PF – 63.81±31.52 years, P<0.05. Females constituted 75.61% and 61.90% in PV and PF group, respectively. Majority of patients with PV were residents of urban and these with PF – of rural areas (70.73% and 66.67%, respectively). On admission, 14 patients with PV (34.15%) and 11 with PF (52.38%) had more than 30% of body surface area involved. In 22 (53.66%) PV mucous membrane (oropharyngeal cavity) was involved. Ten (24.39%) patients with PV and 12 (57.14%) - with PF had more than one concomitant disorder (P<0.05). In treatment prednisone in monotherapy or with additional immunosuppressive agent was mainly used. The disease relapsed within three years after achieving clinical and immunological remission in 29.27% of PV and in 38.10% of PF patients. CONCLUSIONS. PF patients are older than PV ones, more frequently live in rural areas, have more comorbidities. Females constitute majority of pemphigus patients. The disease may relapse in about one third of patients. Because of frequent comorbidities, also these related to pemphigus treatment, patients with pemphigus require complex and multispecialistic medical care.

scholarly journals A Hypothesis Concerning a Potential Involvement of Ceramide in Apoptosis and Acantholysis Induced by Pemphigus Autoantibodies

2010 ◽  
Vol 2010 ◽  
pp. 1-8
Author(s):  
Wendy B. Bollag
Keyword(s):  
Autoimmune Diseases ◽  
Ultraviolet Irradiation ◽  
Cell Adhesion Molecule ◽  
Sun Exposure ◽  
Pemphigus Vulgaris ◽  
Skin Lesions ◽  
Epidermal Keratinocytes ◽  
Pemphigus Foliaceus ◽  
Endemic Pemphigus Foliaceus ◽  
A Cell

Autoimmune diseases affect more than 50 million Americans, resulting in significant healthcare costs. Most autoimmune diseases occur sporadically; however, endemic pemphigus foliaceus (EPF) is an autoimmune skin disease localized to specific geographic loci. EPF, and the related diseases pemphigus vulgaris (PV) and pemphigus foliaceus (PF), are characterized by skin lesions and autoantibodies to molecules found on epidermal keratinocytes. A variant of EPF in patients from El Bagre, Colombia, South America, has recently been reported to be distinct from previously described loci in Brazil and Tunisia epidemiologically and immunologically. As in PF and EPF, El Bagre EPF patients exhibit autoantibodies towards desmoglein-1, a cell adhesion molecule critical for maintaining epidermal integrity. An association of El Bagre EPF with sun exposure has been detected, and ultraviolet irradiation also exacerbates symptoms in PV, PF and EPF. Our hypothesis is that: (1) the autoantibodies generate pathology through an alteration in ceramide metabolism in targeted keratinocytes, resulting in apoptosis and/or cell death and acantholysis, but only when the cell's ability to metabolize ceramide is exceeded, and (2) apoptosis in response to this altered ceramide metabolism is initiated and/or exacerbated by other agents that increase ceramide levels, such as cytokines, ultraviolet irradiation, and senescence.

Drug-induced pemphigus foliaceus with features of pemphigus vulgaris

2001 ◽  
Vol 144 (2) ◽  
pp. 421-422 ◽  
Author(s):  
K. Ogata ◽  
H. Nakajima ◽  
M. Ikeda ◽  
Y. Yamamoto ◽  
M. Amagai ◽  
...  
Keyword(s):  
Pemphigus Vulgaris ◽  
Pemphigus Foliaceus ◽  
Drug Induced

scholarly journals Analysis of clinical characteristics of severe pertussis in infants and children: a retrospective study

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Caiying Wang ◽  
Huimin Zhang ◽  
Yanlan Zhang ◽  
Lin Xu ◽  
Min Miao ◽  
...  
Keyword(s):  
Retrospective Study ◽  
Clinical Characteristics ◽  
Severe Disease ◽  
Severe Pneumonia ◽  
Infants And Children ◽  
Critically Ill Children ◽  
Young Infants ◽  
Incidence And Mortality ◽  
Increasing Trend ◽  
Medical University

Abstract Background The incidence of pertussis shows an increasing trend in recent years, but some clinicians often lack sufficient understanding of the clinical characteristics and risk factors for severe pertussis, and more effective measures should be taken to reduce the incidence and mortality of pertussis in young infants Methods A retrospective study was conducted, and 184 infants and children with pertussis who had been hospitalized in the Department of Pediatrics of Beijing Ditan Hospital affiliated with Capital Medical University from January 2016 to December 2017 were included. Clinical data of the patients were collected and the clinical characteristics were statistically analyzed Results Among the 184 patients, 41.85% were infants < 3 months of age, and 65.22% of the total patients were not vaccinated against pertussis. There were 22 critically ill children, among whom 4 died, and compared with mild cases, they had a higher proportion of children younger than 3 months of age and infants not vaccinated against pertussis (63.64% vs. 38.89% and 100% vs. 60.49%, respectively); a higher proportion of children with severe pneumonia (100% vs. 0%); higher leukocyte count(× 109/L , 35.80 ± 20.53 vs 19.41 ± 8.59); and a higher proportion of children with severe hyperleukocytosis (18.18% vs. 0%, respectively) (P<0.05) Conclusions 1. Infants aged <3 months not vaccinated for pertussis appear more likely to become infected and have more severe disease. 2. Severe pneumonia and hyperleukocytosis are the main mechanisms underlying severe pertussis.

scholarly journals POS0826 SKIN LIMITED IGA VASCULITIS IN ADULTS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 666.1-666
Author(s):  
A. Hočevar ◽  
J. Ostrovrsnik ◽  
K. Perdan-Pirkmajer ◽  
M. Tomsic ◽  
Z. Rotar
Keyword(s):  
Clinical Characteristics ◽  
Systemic Disease ◽  
Elevated Serum ◽  
Skin Lesions ◽  
Current Smoker ◽  
P Value ◽  
Symptom Duration ◽  
Iga Vasculitis ◽  
Reactive Protein

Background:IgA vasculitis (IgAV) could be limited to skin or evolve into a systemic disease, affecting characteristically joints, gastrointestinal tract and/or kidneys.Objectives:We aimed to look for differences between adult IgAV patients with disease limited to skin compared to systemic IgAV.Methods:Medical records of histologically proven adult IgAV cases, diagnosed between January 2010 and December 2020 at our secondary/tertiary rheumatology centre were analyzed.Results:During the 132-month observation period we identified 328 new IgAV cases (59.5% males, median (IQR) age 64.3 (45.1; 76.1) years). Ninety-four (40.2%) patients had skin limited disease, and the rest systemic IgAV.Clinical differences between skin limited and systemic adult IgAV are presented in table 1. Adults with IgAV limited to skin were significantly older, had less commonly skin lesions above the waistline and a lower level of C reactive protein compared to patients with a systemic disease. There were no differences in the frequency of skin necroses between the compared IgAV subgroups. The frequency of potential vasculitis triggers (prior infections, new medications, malignancy) was similar between the compared subgroups.Table 1.Clinical characteristics of IgA vasculitis patients with skin limited and systemic diseaseClinical characteristicsSkin limited IgAV (94)Systemic IgAV (234)P valueMale gender (%)54.361.50.263Age (years)*68.0 (55.0-80.5)61.5 (41.7-75.8)0.007Current smoker (%)13.821.80.123Antecedent infection (%)28.733.80.434New medication23.423.51.0History of cancer12.810.70.569Symptom duration (days)*7 (5-21)8 (5-14)0.756Purpura above waistline36.255.60.002Skin necroses (%)52.145.70.329ESR /mm/h) *32 (18-52)34 (17-53)0.873CRP (g/l) *13.5 (1-32)30 (11-68)<0.001Elevated serum IgA (%)50.649.10.892Legend: * median and IQR;Follow up data were available for 250 (76.2%) patients. During the follow up of median (IQR) 12.5 (6.8 – 22.4) months 35 patients relapsed (13/70 (18.6%) with skin limited IgAV and 22/180 (12.2%) with systemic IgAV, p= 0.224).Conclusion:Skin limited IgAV was associated with older age and less extensive skin puprura in adults. However, relapses of purpura were as common as in systemic IgAV.Disclosure of Interests:None declared

Pemphigus foliaceus in Arabian oryx (Oryx leucoryx)

2021 ◽  
pp. 030098582110305
Author(s):  
Jeffrey B. Curtiss ◽  
Michelle Lewis ◽  
Emily P. Mitchell
Keyword(s):  
Medical Records ◽  
Skin Lesions ◽  
Domestic Animals ◽  
Histological Evaluation ◽  
Pemphigus Foliaceus ◽  
Arid Environments ◽  
Zoological Garden ◽  
Causative Agents ◽  
Arabian Oryx ◽  
Tail Tip

Pemphigus foliaceus (PF) is an autoimmune acantholytic skin disease described in humans, dogs, cats, horses, goats, and sheep. From 2003 to 2016, six Arabian oryx ( Oryx leucoryx) at the National Zoological Garden in Pretoria, South Africa, developed progressive, bilaterally symmetrical, hyperkeratotic skin lesions and pustules consistent with PF. Lesions were similar to those observed in domestic animals and primarily affected the pinnae, face and nasal planum, distal legs, and tail tip. Histological evaluation of suspect PF skin lesions in affected animals, evaluation of medical records for treatments received, causative agents in the diet and environment, and special stains for infectious organisms yielded no consistent inciting cause. The Arabian oryx is a species highly adapted to arid environments of the desert and has recently survived from a severe genetic bottleneck; both of these factors may have contributed to the development of PF in these animals.

scholarly journals On Using a Mobile Application to Support Teledermatology: A Case Study in an Underprivileged Area in Colombia

2018 ◽  
Vol 2018 ◽  
pp. 1-8 ◽  
Author(s):  
Juan Pablo Sáenz ◽  
Mónica Paola Novoa ◽  
Darío Correal ◽  
Bell Raj Eapen
Keyword(s):  
Rural Areas ◽  
Mobile Applications ◽  
Mobile Application ◽  
Correct Diagnosis ◽  
Skin Lesions ◽  
Healthcare Personnel ◽  
Combined Use ◽  
Forest Regions ◽  
The Face

Background. The use of mobile applications in dermatology to support remote diagnosis is gaining acceptance, particularly in rural areas, where dermatology services are commonly managed by healthcare personnel with no specialty training. Moreover, ontologies—sets of concepts that represent knowledge in a given domain—are increasingly being used to support medical diagnosis. A specific case is ONTODerm: an ontology to aid dermatological diagnosis. However, there is little information on the combined use of mobile applications and ontologies as support solutions in dermatology. Objective. Assessing the reliability of ONTODerm as a tool to support remote dermatological diagnosis when used together with a mobile dermatological application in underprivileged areas. Methods. A mobile application that allows characterization of skin lesions was developed, and the information about the lesions was sent to ONTODerm. An exploratory study was conducted in a remote area without access to a dermatologist. A total of 64 dermatological queries were recorded in the application and consulted with ONTODerm. Later, an experienced dermatologist evaluated the characterization and diagnosis of each query to determine the accuracy of the system. Results. The results showed that the probability of obtaining a correct diagnosis was between 64.4% and 85.6% with a confidence interval of 95%. A higher accuracy rate was obtained when the skin lesion occurred on the face or when its border was categorized as poorly demarcated. Conclusions. This study demonstrates the implementation of a teledermatology strategy based on mobile applications and domain ontology-driven knowledge base to provide timely assistance to healthcare professionals. This approach was found to be pertinent in the Colombian rural context, particularly in forest regions, where dermatology specialists are not available. The results of this article do not represent a final validation of the proposed approach; they suggest how the ontology can be improved to effectively support medical staff in marginalized regions.

Coexistence of IgA antibodies to desmogleins 1 and 3 in pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus

2007 ◽  
Vol 156 (4) ◽  
pp. 635-641 ◽  
Author(s):  
L.F. Mentink ◽  
M.C.J.M. de Jong ◽  
G.J. Kloosterhuis ◽  
J. Zuiderveen ◽  
M.F. Jonkman ◽  
...  
Keyword(s):  
Pemphigus Vulgaris ◽  
Pemphigus Foliaceus ◽  
Paraneoplastic Pemphigus ◽  
Iga Antibodies
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