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Generalized Pustular Psoriasis

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https://www.readbyqxmd.com/read/29333670/pustular-psoriasis-and-related-pustular-skin-diseases
#1
REVIEW
H Bachelez
Patients with pustular psoriasis or related pustular diseases may have genetic abnormalities impairing the function of key players of the innate skin immune system. Recently, identification of these abnormalities has changed the paradigm of several of these diseases. These include generalized pustular psoriasis, palmoplantar pustular psoriasis and acrodermatitis continua of Hallopeau, and also drug-induced acute exanthematous generalized pustular eruption. Identified mutations in IL36RN, CARD14 and AP1S3 in different groups of patients lead to enhanced inflammatory cascade in several cellular subtypes including keratinocytes, and to the recruitment and activation of neutrophils and macrophages...
January 15, 2018: British Journal of Dermatology
https://www.readbyqxmd.com/read/29288651/unopposed-il-36-activity-promotes-clonal-cd4-t-cell-responses-with-il-17a-production-in-generalized-pustular-psoriasis
#2
Akiko Arakawa, Sigrid Vollmer, Petra Besgen, Adrian Galinski, Burkhard Summer, Yoshio Kawakami, Andreas Wollenberg, Klaus Dornmair, Michael Spannagl, Thomas Ruzicka, Peter Thomas, Jörg C Prinz
Generalized pustular psoriasis (GPP) is the most severe psoriasis variant. Mutations in the IL-36 antagonist IL36RN, in CARD14 or AP1S3 provide genetic evidence for autoinflammatory aetiology but cannot explain its pathogenesis completely. Here we demonstrate that unopposed IL-36 signalling promotes antigen-driven and likely pathogenic T-helper 17 (Th17) responses in GPP. We observed that CD4+ T cells in blood and skin lesions of GPP patients were characterized by intense hyperproliferation, production of the GPP key mediator, IL-17A, and highly restricted T-cell receptor (TCR) repertoires with identical T-cell clones in blood and skin lesions indicating antigen-driven T-cell expansions...
December 27, 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/29226159/immunohistopathological-findings-of-severe-cutaneous-adverse-drug-reactions
#3
REVIEW
Mari Orime
Diagnosis of severe cutaneous adverse drug reactions should involve immunohistopathological examination, which gives insight into the pathomechanisms of these disorders. The characteristic histological findings of erythema multiforme (EM), Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN) provide conclusive evidence demonstrating that SJS/TEN can be distinguished from EM. Established SJS/TEN shows full-thickness, extensive keratinocyte necrosis that develops into subepidermal bullae. Drug-induced hypersensitivity syndrome (DIHS) and exanthema in drug reaction with eosinophilia and systemic symptoms (DRESS) each display a variety of histopathological findings, which may partly correlate with the clinical manifestations...
2017: Journal of Immunology Research
https://www.readbyqxmd.com/read/29215143/generalized-pustular-psoriasis-in-a-92-year-old-man-with-a-homozygous-nonsense-mutation-in-il36rn
#4
Yoshitaka Ueda, Mayumi Komine, Koji Kamiya, Hidetoshi Tsuda, Takeo Maekawa, Satoru Murata, Mamitaro Ohtsuki
A 92-year-old man developed an erythematous eruption on the trunk and extremities with numerous pustules accompanied by fever. He had never experienced pustular eruption or been diagnosed with psoriasis previously. Skin biopsy revealed Kogoj's spongiform pustule, and he was diagnosed with generalized pustular psoriasis (GPP). Genomic DNA was extracted from his peripheral blood and the sequence of IL36RN gene was analyzed, which revealed a p.Arg10X homozygous mutation. Several cases of elderly-onset GPP have been reported, however, this is the oldest case of GPP...
December 7, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/29186241/clinical-features-of-von-zumbusch-type-of-generalized-pustular-psoriasis-in-children-a-retrospective-study-of-26-patients-in-southwestern-china
#5
Qian Wang, Wei Liu, Lixia Zhang
BACKGROUND: Von Zumbusch type of generalized pustular psoriasis is a rare variant of psoriasis in children. It can occur in patients with or without psoriasis vulgaris. OBJECTIVE: The aim of the study was to discuss the precipitating factors, clinical manifestations, laboratory data and therapy of von Zumbusch type of generalized pustular psoriasis in children from southwestern China and to improve the diagnosis and treatment level. METHODS: A retrospective analysis was conducted for inpatients aged 14 years old or less with von Zumbusch type of generalized pustular psoriasis in our department from 2005 to 2014...
May 2017: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/29168210/abrupt-generalized-pustules-in-patients-with-rheumatoid-arthritis-and-interstitial-lung-disease
#6
Wei Deng, Chaoyang Miao, Xiaoyan Zhang
We report a case of a 30-year-old Chinese woman with rheumatoid arthritis and interstitial lung disease who abruptly developed generalized pustules and a high fever for 10 days. She had been taking oral prednisone, iguratimod and total glucosides of peony regularly for 5 months prior. In addition, she had taken metronidazole for 3 days 20 days prior which she had used before with no adverse reaction. She had no history of similar lesions and psoriasis. A biopsy of a pustule on the back showed spongiform pustule of Kogoj...
November 23, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/29115687/epidemiological-survey-from-2009-to-2012-of-psoriatic-patients-in-japanese-society-for-psoriasis-research
#7
Toshihiro Ito, Hidetoshi Takahashi, Akira Kawada, Hajime Iizuka, Hidemi Nakagawa
Since 1982, the Japanese Society for Psoriasis Research has conducted annual epidemiological surveys of patients with psoriasis. Kawada et al. have reported data for 1982-2001 and Takahashi et al. have reported data for 2002-2008. The present study evaluated 9290 psoriatic cases according to age and sex (2009-2012). The male : female ratio was 2.08:1 (6281 male patients [67.6%] to 3009 female patients [32.4%]). The most prevalent type was psoriasis vulgaris (85.6% of all cases), which was followed by psoriasis arthropathica (6...
November 8, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/29098909/efficacy-and-safety-of-acitretin-monotherapy-in-children-with-pustular-psoriasis-results-from-15-cases-and-a-literature-review
#8
Pingjiao Chen, Changxing Li, Rujun Xue, Huiheng Chen, Xin Tian, Kang Zeng, Xibao Zhang, Jingyao Liang
BACKGROUND: There is a few evidence-based information regarding the efficacy and safety of acitretin treatment in children with pustular psoriasis (PP). OBJECTIVE: This study aimed to provide an additional evidence for this field. METHODS: A retrospective study was undertaken for 15 children with PP, received acitretin in doses of 0.6-1.0 mg/kg/day for 4-6 weeks, the transition dose in 0.2-0.4mg/kg/day for 4-6 weeks and maintenance dose of 0...
November 3, 2017: Journal of Dermatological Treatment
https://www.readbyqxmd.com/read/29034298/generalized-pustular-psoriasis-treated-with-apremilast-in-a-patient-with-multiple-medical-comorbidities
#9
Caleb Jeon, Mio Nakamura, Sahil Sekhon, Di Yan, Jashin J Wu, Wilson Liao, Tina Bhutani
No abstract text is available yet for this article.
November 2017: JAAD Case Reports
https://www.readbyqxmd.com/read/29027743/generalized-pustular-psoriasis-triggered-by-zika-virus-infection
#10
A E Paniz Mondolfi, M Hernandez Perez, G Blohm, M Marquez, A Mogollon Mendoza, C E Hernandez-Pereira, M A Escalona, A Lodeiro Colatosti, J Rothe DeArocha, A J Rodriguez Morales
Zika virus is an emerging arbovirus, which is expanding in epidemic proportions through tropical and subtropical areas of the world. Although Zika is linked to a number of congenital and neurological complications, there is scarce knowledge on the impact of ZIKV infection in human skin. We report the case of a 68-year old woman who presented with generalized pustular psoriasis after a preceding and otherwise uneventful episode of ZIKV infection. Based on recent experimental data on the biology of ZIKV infection in the cutaneous environment, we speculate that ZIKV may have directly triggered the development of generalized pustular psoriasis by stimulation of keratinocyte-derived mediators of inflammation and a polyfunctional T-cell driven immune reaction in the cutaneous milieu...
October 13, 2017: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/28984691/intracorneal-pustular-drug-eruption-a-novel-cutaneous-adverse-event-in-anti-programmed-cell-death-1-patients-that-highlights-the-effect-of-anti-programmed-cell-death-1-in-neutrophils
#11
Cathy Yunjia Zhao, Germana Consuegra, Shaun Chou, Pablo Fernández-Peñas
The introduction of anti-programmed cell death-1 (anti-PD1) monoclonal antibodies has revolutionized the treatment of various advanced malignancies. Despite its efficacy, anti-PD1 therapy is accompanied by a variety of cutaneous adverse events. A 79-year-old man developed erythematous scaly plaques and pustules of the forehead, legs and arms after four cycles of nivolumab infusions every 2 weeks. Histology showed intracorneal pustules with dermal neutrophils and eosinophils. He was treated successfully with topical corticosteroids without discontinuation of nivolumab...
December 2017: Melanoma Research
https://www.readbyqxmd.com/read/28979866/cutaneous-manifestations-of-chikungunya-fever-observations-from-an-outbreak-at-a-tertiary-care-hospital-in-southeast-rajasthan-india
#12
Ramesh Kumar, Manoj K Sharma, Suresh K Jain, Sumit K Yadav, Anil K Singhal
BACKGROUND: Chikungunya fever is caused by chikungunya virus which is transmitted by the bite of infected Aedes aegypti and A. albopictus mosquitoes. AIMS: To study the various mucocutaneous manifestations in suspected cases of chikungunya fever. MATERIALS AND METHODS: The patients who attended our outpatient department from July 2016 to October 2016 and fulfilled the criteria for "suspect cases" of chikungunya infection stipulated by the National Institute of Communicable Diseases, Directorate General of Health Services, Government of India, were included in the study prospectively...
September 2017: Indian Dermatology Online Journal
https://www.readbyqxmd.com/read/28887889/the-genetic-basis-for-most-patients-with-pustular-skin-disease-remains-elusive
#13
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
#14
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
#15
REVIEW
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
#16
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)...
October 2017: MAbs
https://www.readbyqxmd.com/read/28629688/generalized-pustular-psoriasis
#17
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
#18
REVIEW
A A Navarini, A D Burden, F Capon, U Mrowietz, L Puig, S Köks, K Kingo, C Smith, J 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 [Saurat Dermatologie 2016, Rook's Dermatology 2016, Fitzpatrick's 2012 and Braun-Falco 2012], encumbering the collection of phenotypically well-matched groups of patients as well as clinical trials...
November 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
#19
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...
December 2017: Cutaneous and Ocular Toxicology
https://www.readbyqxmd.com/read/28407292/internalized-stigma-in-psoriasis-a-multicenter-study
#20
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
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