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https://www.readbyqxmd.com/read/29150870/acute-and-recurrent-facial-pustulosis-a-unique-clinical-entity
#1
Anthony K Guzman, Lisa K Pappas-Taffer, Dirk M Elston, William D James
We report a series of three patients encountered in a tertiary referral center that share a rare constellation of clinical and histopathologic findings. In each case, a healthy female patient reported recurrent episodes of rapidly-appearing, tightly-clustered, fine pustules on a background of light erythema involving the chin, forehead, cheeks, and neck (Figure 1). These episodic eruptions, which occurred approximately 4-6 episodes per year, were associated with mild pruritus, and relapsed and remitted within 5 days without therapeutic intervention...
November 17, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29143230/the-efficacy-of-biologic-therapy-for-the-management-of-palmoplantar-psoriasis-and-palmoplantar-pustulosis-a-systematic-review
#2
REVIEW
Isabelle M Sanchez, Eric Sorenson, Ethan Levin, Wilson Liao
INTRODUCTION: Palmoplantar psoriasis (PP) and palmoplantar pustulosis (PPP) are diseases affecting the hands and/or feet that can cause marked physical discomfort and functional disability. The tumor necrosis factor-alpha antagonists adalimumab, etanercept, and infliximab, the interleukin (IL)-17A inhibitors ixekizumab and secukinumab, and the IL-23 or IL-12/IL-23 inhibitors guselkumab and ustekinumab have been well studied for the treatment of moderate to severe plaque psoriasis. Less is known about the efficacy and safety of these agents for the treatment of PP (hyperkeratotic and pustular forms) and PPP...
November 15, 2017: Dermatology and Therapy
https://www.readbyqxmd.com/read/29065395/life-threatening-atypical-case-of-acute-generalized-exanthematous-pustulosis
#3
Azadeh Tajmir-Riahi, Petra Wörl, Thomas Harrer, Stefan Schliep, Gerold Schuler, Miklos Simon
Antibiotics are known to cause severe cutaneous adverse reactions, such as the rare acute generalized exanthematous pustulosis (AGEP). Unlike Stevens-Johnson syndrome or toxic epidermal necrolysis, AGEP is rarely life-threatening. Systemic involvement is not typical, and if present usually coincides with a mild elevation of the hepatic enzymes and a decrease in renal function. Hence, AGEP is known to have a good prognosis and to be life-threatening only in elderly patients or patients with chronic diseases...
2017: International Archives of Allergy and Immunology
https://www.readbyqxmd.com/read/29058836/-the-major-scar-syndromes
#4
G E Piérard, M Lesuisse, C Piérard-Franchimont
Some drug eruptions are frequent and follow an indolent course, while others prove to be life-threatening. By contrast, SCAR syndromes are serious skin drug reactions that are rare but their vital prognosis is affected. The three distinct entities of importance are the former Lyell's syndrome, now identified as SJS-TEN syndrome (Stevens-Johnson syndrome/toxic epidermal necrolysis), the DRESS syndrome (drug reaction with eosinophilia and systemic symptoms), and the AGEP syndrome (acute generalized exanthematous pustulosis)...
October 2017: Revue Médicale de Liège
https://www.readbyqxmd.com/read/29057744/acute-localized-exanthematous-pustulosis-due-to-bemiparin
#5
E Gómez Torrijos, M P Cortina de la Calle, Y Méndez Díaz, L Moreno Lozano, A Extremera Ortega, P A Galindo Bonilla, T Alfaya Arias, R García Rodríguez
No abstract text is available yet for this article.
2017: Journal of Investigational Allergology & Clinical Immunology
https://www.readbyqxmd.com/read/29052100/acute-localized-exanthematous-pustulosis-alep-review-of-literature-with-report-of-case-caused-by-amoxicillin-clavulanic-acid
#6
Alessia Villani, Antonello Baldo, Gaia De Fata Salvatores, Vincenzo Desiato, Fabio Ayala, Carlo Donadio
Acute localized exanthematous pustulosis (ALEP) is a localized form of acute generalized exanthematous pustulosis, characterized by acute onset of multiple nonfollicular, pinhead-sized, sterile pustules following drug administration. Antibiotics, especially β-lactams and macrolides, have been implicated in the majority of cases, although eruption after nonsteroidal antiinflammatory drugs and many other medications has also been reported. Skin reaction arises quickly within a few hours, resolving rapidly within a few days without treatment, and it is usually accompanied by fever and neutrophilic leukocytosis...
October 19, 2017: Dermatology and Therapy
https://www.readbyqxmd.com/read/29034454/successful-treatment-of-sapho-syndrome-with-apremilast
#7
S Adamo, J Nilsson, A Krebs, U Steiner, A Cozzio, L E French, A G A Kolios
Synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome is a rare disease with inflammatory osteoarticular and skin involvement. The pathogenesis of SAPHO syndrome remains unclear, but evidence suggests it may be an autoinflammatory disease triggered upon exposure to infectious agents in genetically predisposed individuals. Induction of the IL-23/Th17 axis as well as neutrophil activation seem to play a key role, and therapies targeting these immunological pathways, including TNF-inhibitors, ustekinumab, secukinumab and the IL-1 inhibitor anakinra are potential treatment options that need further investigation...
October 16, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28993983/skin-manifestations-associated-with-autoimmune-liver-diseases-a-systematic-review
#8
REVIEW
Benedetta Terziroli Beretta-Piccoli, Pietro Invernizzi, M Eric Gershwin, Carlo Mainetti
Autoimmune liver diseases, which include mainly autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis, and the variant syndromes, are often associated with extrahepatic autoimmune diseases. However, the association with cutaneous diseases is less well described. In the present article, we provide a systematic literature review on skin manifestations linked to each of these four autoimmune liver diseases, excluding skin manifestations of systemic diseases. The association of autoimmune hepatitis with vitiligo is well known, with a particular striking association with type 2 autoimmune hepatitis, a condition occurring almost entirely in children and adolescents, much rarer and more aggressive than type 1 autoimmune hepatitis; probable associations are also identified with alopecia areata, psoriasis, and pyoderma gangrenosum...
October 9, 2017: Clinical Reviews in Allergy & Immunology
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
#9
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/28977552/synovitis-acne-pustulosis-hyperostosis-osteitis-sapho-paradoxical-reactions-and-different-responses-to-tumour-necrosis-factor-inhibitors
#10
Jean Liew, Teena Huan Xu, Cong-Qiu Chu
No abstract text is available yet for this article.
July 28, 2017: Rheumatology
https://www.readbyqxmd.com/read/28968889/bone-involvement-in-monogenic-autoinflammatory-syndromes
#11
Brigitte Bader-Meunier, Erika Van Nieuwenhove, Sylvain Breton, Carine Wouters
Until recently the most common autoinflammatory diseases (AIDs) associated with bone disease in childhood included a few genetically complex (chronic non-bacterial osteomyelitis, synovitis, acne, pustulosis, hyperostosis and osteitis syndrome) and monogenic (Majeed syndrome, deficiency of IL-1 receptor antagonist, cherubism) AIDs. However, the spectrum of monogenic AIDs associated with bone manifestations has markedly expanded to include both recently identified diseases such as the type I interferonopathies and also newly recognized bone dysplasias in already established AIDs...
August 22, 2017: Rheumatology
https://www.readbyqxmd.com/read/28968231/pustulotic-arthro-osteitis-sonozaki-syndrome-a-rare-case-report
#12
Hatice Resorlu, Besir Sahin Inceer, Sevilay Kılıc, Selda Isık
Pustulotic arthro-osteitis is a rare disease involving the skin and musculoskeletal system that was first described by Sonozaki. Onset is frequently seen at age 30-40. The prevalences between the sexes are similar. Palmoplantar pustulosis and sternoclavicular joint involvement are the most typical findings. It may be difficult to distinguish seronegative spondyloarthropathies and SAPHO syndrome due to sacroiliac joint, vertebral column and peripheral joint involvement. Arthritis being non-erosive and short-lived in character and the absence of deformity or contracture in the joints are significant clinical characteristics...
September 22, 2017: Journal of Back and Musculoskeletal Rehabilitation
https://www.readbyqxmd.com/read/28952335/acitretin-use-in-dermatology
#13
Lyn C Guenther, Rod Kunynetz, Charles W Lynde, R Gary Sibbald, John Toole, Ronald Vender, Catherine Zip
BACKGROUND: Acitretin has been used for the treatment of severe psoriasis for over 20 years. OBJECTIVE: The current project was conceived to optimise patient care by recognising the role acitretin can play in the treatment of patients with psoriasis and those with other disorders of keratinisation. METHODS: A literature review was conducted to explore the role of acitretin and to assess its value for dermatologic disorders other than severe psoriasis...
September 1, 2017: Journal of Cutaneous Medicine and Surgery
https://www.readbyqxmd.com/read/28924111/synovitis-acne-pustulosis-hyperostosis-osteitis-sapho-syndrome-with-significant-bilateral-pleural-effusions
#14
Shoichi Hasegawa, Hiroki Yabe, Naoya Kaneko, Eri Watanabe, Takahisa Gono, Chihiro Terai
We herein report a rare case of a 66-year-old woman who had synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome with marked sternal osteitis and bilateral pleural effusions. SAPHO syndrome was diagnosed based on the characteristic features of a hyperostotic sternum and thoracic spine. The inflammatory changes of sternal osteitis and involvement of the adjacent soft tissue were assumed to be the cause of the pleural effusions. The effusions decreased during the natural course of the disease and resolved after methotrexate therapy...
October 15, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28887889/the-genetic-basis-for-most-patients-with-pustular-skin-disease-remains-elusive
#15
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/28884797/sapho-syndrome-associated-with-hidradenitis-suppurativa-and-pyoderma-gangrenosum-successfully-treated-with-adalimumab-and-methotrexate-a-case-report-and-review-of-the-literature
#16
REVIEW
Dunja A Vekic, Jane Woods, Peter Lin, Geoffrey D Cains
SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome is a rare inflammatory condition describing the combination of skin, bone, and joint manifestations that has a heterogeneous presentation. We report a case of severe SAPHO syndrome in association with hidradenitis suppurativa and pyoderma gangrenosum in a 27-year-old male. The patient had an initial migratory arthritis affecting the knees, ankles, metacarpophalangeal joints, proximal interphalangeal joints, wrists, shoulder, and lower back, which progressed to a persistent arthritis and swelling at the sternum, shoulders, wrists, hands, feet, and lower back...
September 7, 2017: International Journal of Dermatology
https://www.readbyqxmd.com/read/28880693/clinical-features-and-radiological-findings-of-67-patients-with-sapho-syndrome
#17
Hiroshi Okuno, Munenori Watanuki, Yoshiyuki Kuwahara, Akira Sekiguchi, Yu Mori, Shin Hitachi, Keiki Miura, Ken Ogura, Mika Watanabe, Masami Hosaka, Masahito Hatori, Eiji Itoi, Katsumi Sato
OBJECTIVES: The purpose of this study was to facilitate the understanding of the SAPHO (Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis) syndrome by analyzing the clinical and radiological features of 67 Japanese patients with SAPHO syndrome. METHODS: Sixty-seven Japanese patients (female/male: 44/23, mean age at onset: 48.5 years) were diagnosed with SAPHO syndrome from 2002 to 2013 at our hospital. Medical records and radiological imaging of these patients were retrospectively reviewed...
September 7, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/28879224/amicrobial-pustulosis-of-the-folds-and-palmoplantar-pustulosis-simultaneously-induced-by-different-tumor-necrosis-factor-%C3%AE-inhibitors-demonstration-of-a-shared-pathophysiology
#18
Matthew Zirwas, Hershel E Dobkin, Smita Krishnamurthy
No abstract text is available yet for this article.
September 2017: JAAD Case Reports
https://www.readbyqxmd.com/read/28858556/a-case-with-deteriorating-palmoplantar-pustulosis-and-hyperthyroidism-after-simultaneous-bimaxillary-orthognathic-surgery
#19
Aya Oda, Keita Yoshida, Tamayo Uno, Taiga Yoshinaka, Akari Mukai, Masahiro Irifune
A case of palmoplantar pustulosis and hyperthyroidism following orthognathic surgery is presented. Both diseases may have been related to allergic phenomena.
2017: Anesthesia Progress
https://www.readbyqxmd.com/read/28814674/rilonacept-maintains-long-term-inflammatory-remission-in-patients-with-deficiency-of-the-il-1-receptor-antagonist
#20
Megha Garg, Adriana A de Jesus, Dawn Chapelle, Paul Dancey, Ronit Herzog, Rafael Rivas-Chacon, Theresa L Wampler Muskardin, Ann Reed, James C Reynolds, Raphaela Goldbach-Mansky, Gina A Montealegre Sanchez
BACKGROUND: Deficiency of IL-1 receptor antagonist (DIRA) is a rare autoinflammatory disease that presents with life-threatening systemic inflammation, aseptic multifocal osteomyelitis, and pustulosis responsive to IL-1-blocking treatment. This study was performed (a) to investigate rilonacept, a long-acting IL-1 inhibitor, in maintaining anakinra-induced inflammatory remission in DIRA patients, (b) to determine doses needed to maintain remission, and (c) to evaluate the safety and pharmacokinetics of rilonacept in young children (<12 years)...
August 17, 2017: JCI Insight
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