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Generalized Pustular Psoriasis

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https://www.readbyqxmd.com/read/28887889/the-genetic-basis-for-most-patients-with-pustular-skin-disease-remains-elusive
#1
R Mössner, D Wilsmann-Theis, V Oji, P Gkogkolou, S Löhr, P Schulz, A Körber, J Christoph-Prinz, R Renner, K Schäkel, L Vogelsang, K-P Peters, S Philipp, K Reich, H Ständer, A Jacobi, A Weyergraf, K Kingo, S Kõks, S Gerdes, K Steinz, T Schill, K G Griewank, M Müller, S Frey, L Ebertsch, S Uebe, M Sticherling, H Sticht, U Hüffmeier
BACKGROUND: Rare variants in the genes IL36RN, CARD14 and AP1S3 have been identified to cause/ contribute to pustular skin diseases, primarily generalized pustular psoriasis (GPP). OBJECTIVES: To better understand the disease-relevance of these genes, we screened our cohorts of patients with pustular skin diseases (primarily GPP and palmoplantar pustular psoriasis [PPP]) for coding changes in these three genes. Carriers of single heterozygous IL36RN mutations were screened for a second mutation in IL36RN...
August 5, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28776328/acute-generalized-exanthematous-pustulosis-and-polyarthritis-associated-with-a-novel-card14-mutation
#2
Sebastian Podlipnik, Raul Castellanos-Moreira, Helena Florez-Enrich, Juan Ignacio Arostegui, José Manuel Mascaró
Acute generalised exanthematous pustulosis (AGEP) is a rare toxicoderma characterised by an acute onset rash, with many sterile pustules on the surface, high fever and increased acute phase reactants. We report the case of a patient who presented to the dermatology department with an AGEP and polyarthritis, in which a novel CARD14 mutation was identified. The pathophysiological mechanism of AGEP remains unclear, although mutations in the IL36RN gene have been identified in a small subset of AGEP patients. Similarly, mutations in the CARD14 gene have been linked to pustular types of psoriasis and familiar cases of pityriasis rubra pilaris; however, there are no reports associating mutations in the CARD14 gene with AGEP...
August 3, 2017: Australasian Journal of Dermatology
https://www.readbyqxmd.com/read/28747603/autoinflammatory-diseases-in-dermatology-ditra-and-camps
#3
Kazumitsu Sugiura
  Deficiency of interleukin thirty-six receptor antagonist (DITRA) and CARD14 mediated psoriasis (CAMPS) are autoinflammatory diseases in dermatology. The causative genes of DITRA and CMAPS have been identified recently. In this paper, IL36RN and CARD14, the causative gene for DITRA and CAMPS, respectively were explained. In addition, clinical features and therapies for generalized pustular psoriasis not associated with psoriasis vulgaris (GPP without PsV), and pityriasis rubra pilaris type V (PRP type V) were described...
2017: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28726542/generation-and-functional-characterization-of-anti-human-and-anti-mouse-il-36r-antagonist-monoclonal-antibodies
#4
Rajkumar Ganesan, Ernest L Raymond, Detlev Mennerich, Joseph R Woska, Gary Caviness, Christine Grimaldi, Jennifer Ahlberg, Rocio Perez, Simon Roberts, Danlin Yang, Kavita Jerath, Kristopher Truncali, Lee Frego, Eliud Sepulveda, Priyanka Gupta, Su-Ellen Brown, Michael D Howell, Keith A Canada, Rachel Kroe-Barrett, Jay S Fine, Sanjaya Singh, M Lamine Mbow
Deficiency of interleukin (IL)-36 receptor antagonist (DITRA) syndrome is a rare autosomal recessive disease caused by mutations in IL36RN. IL-36R is a cell surface receptor and a member of the IL1R family that is involved in inflammatory responses triggered in skin and other epithelial tissues. Accumulating evidence suggests that IL-36R signaling may play a role in the pathogenesis of psoriasis. Therapeutic intervention of IL-36R signaling offers an innovative treatment paradigm for targeting epithelial cell-mediated inflammatory diseases such as the life-threatening psoriasis variant called generalized pustular psoriasis (GPP)...
July 20, 2017: MAbs
https://www.readbyqxmd.com/read/28629688/generalized-pustular-psoriasis
#5
Hideyuki Kosumi, Takamasa Ito, Yasuyuki Fujita, Kentaro Izumi, Yuka Maya, Teruki Yanagi, Ken Natsuga, Hideyuki Ujiie, Satoru Shinkuma, Toshifumi Nomura, Naoya Sadanobu, Hiroshi Shimizu
No abstract text is available yet for this article.
September 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28585342/european-consensus-statement-on-phenotypes-of-pustular-psoriasis
#6
REVIEW
Alexander A Navarini, A David Burden, Francesca Capon, Ulrich Mrowietz, Luis Puig, Sulev Köks, Külli Kingo, Catherine Smith, Jonathan N Barker
Pustular psoriasis (PP) is a group of inflammatory skin conditions characterized by infiltration of neutrophil granulocytes in the epidermis to such an extent that clinically visible sterile pustules develop. Because of clinical co-incidence, PP is currently grouped with psoriasis vulgaris (PV). However, PP and PV are phenotypically different, respond differently to treatments, and seem to be distinct on the genetic level. In contrast to PV, the phenotypes of PP are not well defined. Descriptions of each form of PP are discordant among standard dermatology textbooks [1-5], encumbering the collection of phenotypically well-matched groups of patients as well as clinical trials...
June 6, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28412864/cutaneous-allergic-drug-reactions-update-on-pathophysiology-diagnostic-procedures-and-differential-diagnosic
#7
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 27, 2017: Cutaneous and Ocular Toxicology
https://www.readbyqxmd.com/read/28407292/internalized-stigma-in-psoriasis-a-multicenter-study
#8
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
#9
LETTER
C Mugheddu, L Atzori, A Lappi, M Pau, S Murgia, F Rongioletti
No abstract text is available yet for this article.
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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...
April 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28130024/acute-generalized-erythrodermic-pustular-psoriasis-associated-with-bupropion-naltrexone-contrave-%C3%A2
#16
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
#17
REVIEW
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...
May 2017: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28063630/clinical-profiles-of-pediatric-patients-with-gpp-alone-and-with-different-il36rn-genotypes
#18
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...
March 2017: Journal of Dermatological Science
https://www.readbyqxmd.com/read/28043870/il-1-and-il-36-are-dominant-cytokines-in-generalized-pustular-psoriasis
#19
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...
July 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/27990381/psoriasis-epidemiology-clinical-features-co-morbidities-and-clinical-scoring
#20
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
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