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Generalized Pustular Psoriasis

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https://www.readbyqxmd.com/read/28412864/cutaneous-allergic-drug-reactions-update-on-pathophysiology-diagnostic-procedures-and-differential-diagnostic
#1
Galina Balakirski, Hans F Merk
Important changes in the understanding and management of drug hypersensitivity reactions during the last years result from the increasing importance of biologics in medical practice, which differ in their spectrum of adverse drug reactions (ADRs) from the classical covalent drugs. With regard to covalent drugs, ampicillin and amoxicillin as well as clavulanic acid play an increasing role among ADRs to betalactam antibiotics. Fluoroquinolones are mainly the cause of anaphylactic and photosensitivity reactions...
April 17, 2017: Cutaneous and Ocular Toxicology
https://www.readbyqxmd.com/read/28407292/internalized-stigma-in-psoriasis-a-multicenter-study
#2
Erkan Alpsoy, Mualla Polat, Bilge FettahlıoGlu-Karaman, Ayse Serap Karadag, Pelin Kartal-Durmazlar, Basak YalCın, Selma Emre, Didem Didar-Balcı, Asli Bilgic-Temel, Ercan Arca, Rafet Koca, Kamer Gunduz, Murat Borlu, Tulin Ergun, Seval Dogruk-Kacar, Ayca Cordan-Yazici, Pınar Dursun, Ozlem BilgiC, Serap Gunes-Bilgili, Neslihan Sendur, Ozge Baysal, Ibrahim Halil-Yavuz, Gizem Yagcioglu, Ertan Yilmaz, Ufuk Kavuzlu, Yesim Senol
Internalized stigma is the adoption of negative attitudes and stereotypes of the society regarding a person's illness. It causes decreased self-esteem and life-satisfaction, increased depression and suicidality, and difficulty in coping with the illness. The primary aim of this study was to investigate the internalized stigma state of psoriatic patients and to identify the factors influencing internalized stigma. The secondary aim was to identify the correlation of internalized stigma with quality of life and perceived health status...
April 13, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28319281/successful-secukinumab-treatment-of-generalized-pustular-psoriasis-and-erythrodermic-psoriasis
#3
C Mugheddu, L Atzori, A Lappi, M Pau, S Murgia, F Rongioletti
Erythrodermic psoriasis (EP) and generalized pustular psoriasis (GPP) are the most severe forms of psoriasis, resulting in significant morbidity and even mortality (1) . There are few evidence-based data on their treatment (2,3) . Secukinumab, a fully human anti-IL-17A monoclonal antibody, demonstrated strong efficacy in treating moderate to severe plaque-type psoriasis (4,5) . This article is protected by copyright. All rights reserved.
March 20, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28300318/interleukin-17-blockade-in-generalized-pustular-psoriasis-new-hope-for-severely-ill-patients
#4
A Reich
No abstract text is available yet for this article.
March 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28295164/autosomal-dominant-familial-generalized-pustular-psoriasis-caused-by-a-card14-mutation
#5
T Takeichi, A Kobayashi, E Ogawa, Y Okuno, S Kataoka, M Kono, K Sugiura, R Okuyama, M Akiyama
In 2012, gain-of-function mutations in CARD14, which encodes caspase recruitment domain family member 14, were identified as the cause of familial psoriasis vulgaris (PV) and familial pityriasis rubra pilaris (PRP).(1,2) We and another group reported that CARD14 variants are associated with generalized pustular psoriasis (GPP) and palmoplantar pustular psoriasis (PPP).(3-5) The other reports mentioned that CARD14 mutations in individuals with GPP and erythrodermic PRP. (4,6) Very recently, we described PRP type V as an autoinflammatory disease caused by CARD14 mutations...
March 10, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28255119/-clinical-analysis-of-82-cases-of-generalized-pustular-psoriasis
#6
Xiaoqi Wu, Yaping Li
To analyze clinical features, laboratory tests, treatment and prognosis for patients with generalized pustular psoriasis (GPP).
 Methods: Clinical data of 82 patients with GPP (16 cases of children, 66 cases of adults) from the Department of Dermatology, Second Xiangya Hospital, Central South University were retrospectively analyzed.
 Results: Among the GPP patients, the male to female ratio was 1:1.1. Average age of onset in patients without history of psoriasis vulgaris (GPPPSO-) was earlier than that of patients with history of psoriasis vulgaris (GPPPSO+)[(25...
February 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/28196704/toll-like-receptor-4-antagonist-tak-242-inhibits-autoinflammatory-symptoms-in-ditra
#7
Akitaka Shibata, Kazumitsu Sugiura, Yasuhide Furuta, Yoshiko Mukumoto, Osamu Kaminuma, Masashi Akiyama
BACKGROUND: IL36RN encodes the IL-36 receptor antagonist (IL-36Ra), and loss-of-function mutations in IL36RN define a recessively inherited autoinflammatory disease named "deficiency of IL-36Ra" (DITRA). DITRA causes systemic autoinflammatory diseases, including generalized pustular psoriasis (GPP), an occasionally life-threatening disease that is characterized by widespread sterile pustules on the skin, fever and other systemic symptoms. GPP can present at any age, and provocative factors include various infections, medicines and pregnancy...
February 11, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28194751/juvenile-generalized-pustular-psoriasis-is-a-chronic-recalcitrant-disease-an-analysis-of-27-patients-seen-in-a-tertiary-hospital-in-johor-malaysia
#8
Bi-Wen Lau, Dee-Zhen Lim, Francesca Capon, Jonathan N Barker, Siew-Eng Choon
BACKGROUND: Limited information exists regarding juvenile generalized pustular psoriasis (GPP). We aim to determine the clinical profile and outcome of Malaysians with juvenile GPP. METHODS: Review of hospital case notes on patients with juvenile GPP. RESULTS: Twenty-seven patients with juvenile GPP were identified. Female to male ratio was 1.4:1. The median age at onset of GPP was 6.5 years. Ten patients had prior psoriasis with a median pre-pustular duration of 2...
February 14, 2017: International Journal of Dermatology
https://www.readbyqxmd.com/read/28168661/psoriasis-in-systemic-lupus-erythematosus-a-single-center-experience
#9
Konstantinos Tselios, Kristy Su-Ying Yap, Rattapol Pakchotanon, Ari Polachek, Jiandong Su, Murray B Urowitz, Dafna D Gladman
The coexistence of psoriasis with systemic lupus erythematosus (SLE) has been reported in limited case series, raising hypotheses about shared pathogenetic mechanisms. Nevertheless, important differences regarding treatment do exist. The aim of the present study was to determine the prevalence and characteristics of psoriasis in a defined cohort of lupus patients. Patients with psoriasis were retrieved from the University of Toronto Lupus Clinic from its inception in 1970 up to 2015. Charts were hand-searched to collect information concerning demographic, clinical, and therapeutic variables...
February 6, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28130024/acute-generalized-erythrodermic-pustular-psoriasis-associated-with-bupropion-naltrexone-contrave-%C3%A2
#10
Priyanka A Singh, Kerry P Cassel, Ronald M Moscati, David Eckersley
BACKGROUND: We report a case of erythrodermic pustular psoriasis associated with initiation of bupropion/naltrexone (Contrave®; Orexigen Therapeutics, La Jolla, CA) in a patient with no history of psoriasis. CASE REPORT: A 55-year-old woman was transferred to our tertiary medical center from a community hospital for possible Stevens-Johnson syndrome 3 weeks after initiation of bupropion/naltrexone. The patient was admitted to the burn unit for wound treatment and hydration...
April 2017: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28108048/bullous-pseudobullous-pustular-dermatoses
#11
Mark R Wick
Several dermatoses are typified by the formation of spaces (blisters; bullae) within or beneath the epidermis. These may be acellular or filled with particular species of inflammatory cells. Etiological categories include infectious, immune-mediated, genetic, drug-related, and idiopathic lesions. Examples of such disorders include impetigo, Herpes virus infections, pemphigus, bullous pemphigoid and pemphigoid gestationis, epidermolysis bullosa acquisita, IgA-related dermatoses, inherited epidermolysis bullosa variants, Hailey-Hailey disease, and porphyria cutanea tarda...
December 14, 2016: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28063630/clinical-profiles-of-pediatric-patients-with-gpp-alone-and-with-different-il36rn-genotypes
#12
Yirong Wang, Ruhong Cheng, Zhiyong Lu, Yifeng Guo, Ming Yan, Jianying Liang, Peichen Huang, Ming Li, Zhirong Yao
BACKGROUND: IL36RN mutation has been identified as one pathogenesis of generalized pustular psoriasis, but the existence of GPP patients without mutation makes this controversial. OBJECTIVE: Our study aimed at assessing the differences in clinical profiles of children with GPP, with and without IL36RN mutation. METHODS: An ambispective case series study involved review of the records of 66 childhood patients with pediatric-onset GPP and without previous psoriasis vulgaris...
December 13, 2016: Journal of Dermatological Science
https://www.readbyqxmd.com/read/28043870/il-1-and-il-36-are-dominant-cytokines-in-generalized-pustular-psoriasis
#13
Andrew Johnston, Xianying Xing, Liza Wolterink, Drew H Barnes, ZhiQiang Yin, Laura Reingold, J Michelle Kahlenberg, Paul W Harms, Johann E Gudjonsson
BACKGROUND: Generalized pustular psoriasis (GPP) is a rare, debilitating, and often life-threatening inflammatory disease characterized by episodic infiltration of neutrophils into the skin, pustule development, and systemic inflammation, which can manifest in the presence or absence of chronic plaque psoriasis (PV). Current treatments are unsatisfactory and warrant a better understanding of GPP pathogenesis. OBJECTIVE: We sought to understand better the disease mechanism of GPP to allow improved targeted therapies...
December 31, 2016: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/27990381/psoriasis-epidemiology-clinical-features-co-morbidities-and-clinical-scoring
#14
Sunil Dogra, Rahul Mahajan
On the basis of current evidence derived from hospital-based studies, mostly from North India, the prevalence of psoriasis in adults varies from 0.44 to 2.8%, with a much lower prevalence in children. The peak age at onset in adults is in the third and fourth decade of life, with a slight male preponderance. It is recommended that population-based large epidemiologic studies should be undertaken in different parts of the country for estimating the correct prevalence of psoriasis in general population. Chronic plaque-type psoriasis is the most common morphologic presentation of psoriasis, accounting for more than 90% of all cases...
November 2016: Indian Dermatology Online Journal
https://www.readbyqxmd.com/read/27915962/generalized-pustular-psoriasis-von-zumbusch-type-following-renal-transplant-a-case-report
#15
Georgia Dedemadi, Maria Koukoulaki, Vassileios Vougas, Constantinos Noutsis, Aikaterini Pantelidaki, Spiros Drakopoulos
Generalized pustular psoriasis appears as an uncommon variant form of psoriasis consisting of widespread pustules on an erythematous background (von Zumbusch). A 39-year-old male patient with a history of plaque psoriasis since the age of 9 who had an acute relapse of generalized pustular psoriasis 12 days following a successful renal transplantation is presented. Despite administered immunosuppression for transplantation, the addition of cyclosporine and methotrexate did not reverse the ongoing process of disease and the patient died on the 57th posttransplant day due to multiorgan failure subsequent to severe bone marrow suppression...
December 2, 2016: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/27904292/a-case-of-acrodermatitis-continua-accompanying-with-osteolysis-and-atrophy-of-the-distal-phalanx-that-evoluted-into-generalized-pustular-psoriasis
#16
Kyung Ho Kim, Hong Lim Kim, Hyun Yi Suh, Jae Wook Jeon, Ji Young Ahn, Mi Youn Park, Jai Il Youn
No abstract text is available yet for this article.
December 2016: Annals of Dermatology
https://www.readbyqxmd.com/read/27900482/mutations-in-il36rn-are-associated-with-geographic-tongue
#17
Jianying Liang, Peichen Huang, Huaguo Li, Jia Zhang, Cheng Ni, Yirong Wang, Jinwen Shen, Chunxiao Li, Lu Kang, Jie Chen, Hui Zhang, Zhen Wang, Zhen Zhang, Ming Li, Zhirong Yao
Geographic tongue (GT) is a benign inflammatory disorder of unknown etiology. Epidemiology and histopathology in previous studies found that generalized pustular psoriasis (GPP) is a factor associated with GT, but the molecular mechanism remains obscure. To investigate the mechanism of GT, with and without GPP, three cohorts were recruited to conduct genotyping of IL36RN, which is the causative gene of GPP. In a family spanning three generations and diagnosed with only GT ("GT alone"), GT was caused by the c...
February 2017: Human Genetics
https://www.readbyqxmd.com/read/27884600/six-transmembrane-epithelial-antigens-of-the-prostate-comprise-a-novel-inflammatory-nexus-in-patients-with-pustular-skin-disorders
#18
Yun Liang, Xianying Xing, Maria A Beamer, William R Swindell, Mrinal K Sarkar, Liza Wolterink Roberts, John J Voorhees, J Michelle Kahlenberg, Paul W Harms, Andrew Johnston, Johann E Gudjonsson
BACKGROUND: Pustular skin disorders are a category of difficult-to-treat and potentially life-threatening conditions that involve the appearance of neutrophil-rich pustules. The molecular basis of most pustular skin conditions has remained unknown. OBJECTIVE: We sought to investigate the molecular basis of 3 pustular skin disorders: generalized pustular psoriasis (GPP), palmoplantar pustulosis (PPP), and acute generalized exanthematous pustulosis (AGEP). METHODS: Microarray analyses were performed to profile genome-wide gene expression of skin biopsy specimens obtained from patients with GPP, PPP, or AGEP and healthy control subjects...
April 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/27828647/generalized-pustular-psoriasis-induced-by-systemic-steroid-dose-reduction
#19
Danielle Cristine Westphal, Antonio Pedro Mendes Schettini, Petra Pereira de Souza, Jessica Castiel, Carlos Alberto Chirano, Mônica Santos
Generalized pustular psoriasis, or psoriasis of von Zumbusch, is an acute and severe clinical form of psoriasis, which usually occurs in patients with psoriasis undergoing aggravating factors. In this work, we report the case of a female patient, 70 years old, who developed generalized pustular psoriasis symptoms while reducing the dose of oral corticosteroids, improperly introduced for the treatment of alleged acute generalized exanthematous pustulosis. The differential diagnosis of generalized pustular psoriasis should be made with other pustular dermatoses, such as subcorneal pustulosis, IgA pemphigus and especially with acute generalized exanthematous pustulosis...
September 2016: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/27795221/-acute-generalized-exanthematous-pustulosis-induced-by-piroxicam
#20
Ingrid Bissinger, Gustavo Matute-Turizo, María Natalia Mejía-Barreneche
BACKGROUND: Between 62 and 90% of cases of acute generalized exanthematous pustulosis are caused by drugs. Its onset is rapid with generalized pustules, fever, and blood neutrophil count over 7000; pustules resolve spontaneously in less than 15 days. A case associated with piroxicam described. CASE REPORT: A 36-year-old with initial erythema of the thorax and abdomen, accompanied by burning, without fever, which later spread to his forearms, upper arms, and thighs, with face edema...
October 2016: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
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