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https://www.readbyqxmd.com/read/28814674/rilonacept-maintains-long-term-inflammatory-remission-in-patients-with-deficiency-of-the-il-1-receptor-antagonist
#1
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
https://www.readbyqxmd.com/read/28796198/in-vitro-assays-in-severe-cutaneous-adverse-drug-reactions-are-they-still-research-tools-or-diagnostic-tests-already
#2
Grzegorz Porebski
Severe cutaneous adverse drug reactions (SCARs) represent life-threatening medical conditions and an appropriate causative diagnosis of these conditions is of the highest importance. Existing in vivo diagnostic methods are risky or are just contraindicated in these patients. Therefore, in vitro tests take on greater significance. In this survey, the studies on in vitro assays in SCARs were identified with a defined searching strategy and strict eligibility criteria. Different methods in the particular clinical manifestations and the groups of drugs were compared in respect to the diagnostic parameters obtained...
August 10, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28776328/acute-generalized-exanthematous-pustulosis-and-polyarthritis-associated-with-a-novel-card14-mutation
#3
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/28768672/an-illusion-of-septic-shock-acute-generalised-exanthematous-pustulosis-with-multiorgan-dysfunction
#4
Manisha Jakkidi, Carine Basmadjian, Satyajeet Roy
Acute generalised exanthematous pustulosis (AGEP) is a rare cutaneous eruption, most often caused by commonly used antibiotics. It is characterised by an acute onset of non-follicular sterile pustular rash and erythema within hours or days of drug exposure and usually resolves spontaneously within 1-2 weeks once the drug is discontinued. Haemodynamic involvement in the form of shock is rare. Here, we present a severe case of AGEP, manifesting with systemic involvement and haemodynamic instability resulting in shock with multiorgan dysfunction...
August 2, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28765183/a-case-of-bronchiolitis-obliterans-organising-pneumonia-associated-with-sapho-synovitis-acne-pustulosis-hyperostosis-osteitis-syndrome
#5
Fawad Hameed, Henry Steer
A 57-year-old woman with SAPHO (synovitis-acne-pustulosis-hyperostosis-osteitis) syndrome presented with recurrent episodes of pneumonia. She was treated with multiple courses of antibiotics with no success. The transbronchial biopsy undertaken via bronchoscopy revealed organising pneumonia (OP). She was treated with steroids and responded well with full clinical recovery and normalisation of her chest X-ray.To our knowledge, this is the first reported case of OP in association with SAPHO syndrome. This case report highlights the importance of considering OP in patients with SAPHO syndrome who present with chest infection...
August 1, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28764899/clinical-features-etiologic-factors-associated-disorders-and-treatment-of-palmoplantar-pustulosis-the-mayo-clinic-experience-1996-2013
#6
Jeannette M Olazagasti, Janice E Ma, David A Wetter
OBJECTIVE: To further characterize clinical characteristics, etiologic factors, associated disorders, and treatment of palmoplantar pustulosis (PPP). PATIENTS AND METHODS: We conducted a retrospective review of patients with PPP at Mayo Clinic between January 1, 1996, and December 31, 2013. RESULTS: Of 215 patients with PPP identified, 179 (83%) were female, and the mean age at onset was 45.3 years. Most patients (n=165, 77%) were current or former smokers...
July 29, 2017: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/28725947/clinical-heterogeneity-of-sapho-syndrome-challenging-diagnose-and-treatment
#7
REVIEW
Francesco Cianci, Angelo Zoli, Elisa Gremese, Gianfranco Ferraccioli
Synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome is a rare disease which is often misdiagnosed and under-recognized, because of its peculiar and heterogeneous clinical presentation. Its main features consist of cutaneous and osteoarticular manifestations, the latter affecting more often the anterior chest wall and having typical radiologic findings. There are no validated diagnostic criteria for SAPHO and no guidelines for treatment, due mainly to its rarity; as a consequence, therapy is empirical and aimed to control pain and modifying inflammatory process...
July 19, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28715916/drug-management-of-neutrophilic-dermatoses
#8
Simone Garcovich, Clara De Simone, Emilio Berti, Angelo Valerio Marzano
Neutrophilic dermatoses are a heterogenous group of chronic, cutaneous inflammatory conditions characterized by the accumulation of neutrophils in the skin and by systemic inflammation. Neutrophilic dermatoses can be idiopathic or associated with other inflammatory or systemic diseases, including the group of the hereditary, autoinflammatory syndromes. Clinical management is challenging, due to limited clinical evidence and lack of clinical practice guidelines. Areas covered: This review provides an overview of current therapeutic management of the three prototypical neutrophilic dermatoses, aseptic pustulosis of the folds, Sweet syndrome and pyoderma gangrenosum...
July 27, 2017: Expert Review of Clinical Pharmacology
https://www.readbyqxmd.com/read/28704988/acute-generalized-exanthema-tous-pustulosis-agep-associated-to-epstein-barr-virus-ebv-infection-reactivation
#9
Simona Giancristoforo, Andrea Diociaiuti, Diana Mennis, Cristina Rota, Cristina Russo, Luana Coltella, Mauro Paradisi, May El Hachem
No abstract text is available yet for this article.
July 11, 2017: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
https://www.readbyqxmd.com/read/28703347/non-pigmenting-fixed-drug-eruption-with-mixed-features-of-acute-generalized-exanthematous-pustulosis-induced-by-pseudoephedrine-a-case-report
#10
Risa Fukuda, Takeshi Ouchi, Ikuko Hirai, Takeru Funakoshi, Aki Honda, Keiji Tanese, Akiko Tanikawa, Masayuki Amagai, Hayato Takahashi
No abstract text is available yet for this article.
August 2017: Contact Dermatitis
https://www.readbyqxmd.com/read/28699044/uva1-vs-narrowband-uvb-phototherapy-in-the-treatment-of-palmoplantar-pustulosis-a-pilot-randomized-controlled-study
#11
Li-Na Su, Jie Ren, Shi-Meng Cheng, Jian-Lan Liu, Yang-Feng Ding, Ning-Wen Zhu
UVA1 phototherapy, a new therapeutic approach, has recently been shown good efficacy in the treatment of palmoplantar pustulosis (PPP). The purpose of this study was to compare the efficacy of UVA1 and narrowband UVB (NB-UVB) therapy in the treatment of PPP. Patients with PPP were randomly assigned to either UVA1 or NB-UVB therapy according to a left-right randomization table. Both treatments were performed three times weekly for up to 30 sessions. Clinical evaluation was based on the Palmoplantar Pustular Psoriasis Area and Severity Index (PPPASI) score...
July 11, 2017: Lasers in Medical Science
https://www.readbyqxmd.com/read/28688013/a-comprehensive-review-of-neutrophilic-diseases
#12
REVIEW
Angelo V Marzano, Alessandro Borghi, Daniel Wallach, Massimo Cugno
Neutrophilic dermatoses are a group of conditions characterized by the accumulation of neutrophils in the skin and clinically presenting with polymorphic cutaneous lesions, including pustules, bullae, abscesses, papules, nodules, plaques and ulcers. In these disorders, the possible involvement of almost any organ system has lead to coin the term 'neutrophilic diseases'. Neutrophilic diseases have close clinicopathological similarities with the autoinflammatory diseases, which present with recurrent episodes of inflammation in the affected organs in the absence of infection, allergy and frank autoimmunity...
July 7, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28670258/insulin-resistance-diabetes-mellitus-and-thyroid-dysfunction-in-patients-with-palmoplantar-pustulosis-a-case-controlled-study
#13
Hatice Ataş, Müzeyyen Gönül
INTRODUCTION: Palmoplantar pustulosis (PPP) is a chronic pustular inflammatory skin disease; however, its pathogenesis is not well understood. Several factors, such as genetics, tobacco use and autoimmune issues, may contribute to this disease. AIM: This research was conducted to investigate the relationships between insulin resistance, thyroid disease and PPP. MATERIAL AND METHODS: Thirty-three patients with PPP and 27 age- and gender-matched controls were analysed for their smoking histories, thyroid function tests, anti-thyroid peroxidase antibody (anti-TPO) levels, fasting glucose, fasting insulin levels and the homeostatic model assessment (HOMA) index for insulin resistance...
June 2017: Postȩpy Dermatologii i Alergologii
https://www.readbyqxmd.com/read/28634314/-a-case-of-moyamoya-disease-associated-with-sapho-syndrome
#14
Nobutaka Horie, Mayuko Baba, Ken Kawada, Yuuki Matsunaga, Eisaku Sadakata, Yoichi Morofuji, Tsuyoshi Izumo, Minoru Morikawa, Takeo Anda, Takayuki Matsuo
Moyamoya disease is a unique occlusive disease of the internal carotid artery(ICA)with moyamoya vessels that can lead to transient ischemic attacks and hemorrhagic stroke. When other inherited or acquired disorders and conditions occur in conjunction with moyamoya disease, the syndrome is known as quasi-moyamoya disease. We report the case of a 34-year-old woman with a past history of SAPHO(Synovitis-Acne-Pustulosis-Hyperostosis-Osteomyelitis)syndrome, who presented with arm weakness and headache. Magnetic resonance angiography revealed severe terminal stenosis of the left ICA with moyamoya vessels, and she was diagnosed as moyamoya disease associated with SAPHO syndrome...
June 2017: No Shinkei Geka. Neurological Surgery
https://www.readbyqxmd.com/read/28617679/bendamustine-induced-acute-generalized-exanthematous-pustulosis-confirmed-by-patch-testing
#15
Ira Daniel Harber, Kristen V Adams, Kathleen Casamiquela, Stephen Helms, Brandon T Benson, Vince Herrin
No abstract text is available yet for this article.
July 2017: Dermatitis
https://www.readbyqxmd.com/read/28611628/overlapping-dress-and-stevens-johnson-syndrome-case-report-and-review-of-the-literature
#16
Aneline Casagranda, Mariano Suppa, Florence Dehavay, Véronique Del Marmol
Drug-induced severe cutaneous adverse reactions (SCARs) include acute generalized exanthematous pustulosis, drug reaction with eosinophilia and systemic symptoms (DRESS), and epidermal necrolysis (Stevens-Johnson syndrome [SJS], toxic epidermal necrolysis). The identification of the causal drug is crucial in order to avoid further exposure, but making the right differential diagnosis of the type of SCAR is equally important since treatment, follow-up, and prognosis of different SCARs are not the same. These syndromes are distinct entities with different clinical, biological, and histological patterns, but sometimes the early distinction between 2 SCARs can be extremely challenging, and overlapping conditions could therefore be taken into consideration, although true overlapping SCARs are very rare when using strict diagnostic criteria (described by the RegiSCAR group)...
May 2017: Case Reports in Dermatology
https://www.readbyqxmd.com/read/28597181/clinical-signs-pathophysiology-and-management-of-cutaneous-side-effects-of-anti-tumor-necrosis-factor-agents
#17
REVIEW
Siegfried Segaert, Caroline Hermans
Approximately one in four patients treated with anti-TNF agents (infliximab, etanercept, adalimumab, certolizumab, and golimumab) develops cutaneous adverse events, typically months to years after the initiation of treatment, with xerosis cutis, eczema (often psoriasiform), psoriasis, palmoplantar pustulosis, cutaneous infections, alopecia, and skin cancer being the most frequently encountered. The typical skin lesion of anti-tumor necrosis factor (TNF)-treated patients is orange-red psoriasiform eczema affecting the flexures, genitalia, scalp, or face, with high susceptibility to bacterial superinfection with Staphylococcus aureus...
June 8, 2017: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/28585342/european-consensus-statement-on-phenotypes-of-pustular-psoriasis
#18
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/28579754/paradoxical-sapho-syndrome-observed-during-anti-tnf%C3%AE-therapy-for-crohn-s-disease
#19
Hitoshi Amano, Reikei Matsuda, Tomohiko Shibata, Daisuke Takahashi, Shinichiro Suzuki
Currently, anti-TNFα antibodies are used to treat Crohn's disease. We report on a 45-year-old Japanese female with Crohn's disease developing SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome following exposure to the anti-TNFα antibody adalimumab. Initially, adalimumab induced remission, but the patient showed SAPHO syndrome 11 weeks following the start of adalimumab therapy for the first time. Cutaneous and articular involvement were exacerbating the condition, so adalimumab was discontinued and the patient was put on low-dose methotrexate to control her symptoms...
2017: Biologics: Targets & Therapy
https://www.readbyqxmd.com/read/28559812/rapid-involution-of-pustules-during-topical-steroid-treatment-of-acute-generalized-exanthematous-pustulosis
#20
Christiane Kley, Carla Murer, Julia-Tatjana Maul, Barbara Meier, Florian Anzengruber, Alexander A Navarini
Acute generalized exanthematous pustulosis (AGEP) is a dramatic generalized pustular rash of severe onset, which is considered a serious cutaneous adverse reaction to drugs. However, even though the clinical features are impressive and are often accompanied by systemic inflammation, it can be controlled quickly and safely by topical steroids subsequent to interruption of the offending drug. Here, we describe the management of a case and the evolution of the pustular rash. An elderly woman consulted with a generalized crop of 2-3 mm, nonfollicular pustules on erythematous background...
January 2017: Case Reports in Dermatology
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