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Quoted phrase not found in phrase index: "Autoinflammatory syndrome with skin involvement"
Page 1
Immunopathogenesis of Behçet's disease and treatment modalities.
van der Houwen TB, van Hagen PM, van Laar JAM. van der Houwen TB, et al. Semin Arthritis Rheum. 2022 Feb;52:151956. doi: 10.1016/j.semarthrit.2022.151956. Epub 2022 Jan 10. Semin Arthritis Rheum. 2022. PMID: 35038644 Free article. Review.
The inflammation causes a typical clinical phenotype including orogenital ulcerations, uveitis and skin lesions. Treatment is aimed at the aberrations found in the innate (neutrophils and gammadelta-T cells) and adaptive immune system (TNF-alpha, INF-gamma, IL-1), d …
The inflammation causes a typical clinical phenotype including orogenital ulcerations, uveitis and skin lesions. Treatment is …
Cold urticaria - What we know and what we do not know.
Maltseva N, Borzova E, Fomina D, Bizjak M, Terhorst-Molawi D, Košnik M, Kulthanan K, Meshkova R, Thomsen SF, Maurer M; COLD-CE Steering Committee. Maltseva N, et al. Allergy. 2021 Apr;76(4):1077-1094. doi: 10.1111/all.14674. Epub 2020 Dec 24. Allergy. 2021. PMID: 33249577 Review.
Recent research and guideline updates have advanced our understanding and management of ColdU. Today, its pathophysiology is thought to involve the cold-induced formation of autoallergens and IgE to these autoallergens, which provoke a release of proinflammatory mediators …
Recent research and guideline updates have advanced our understanding and management of ColdU. Today, its pathophysiology is thought to i …
Somatic Mutations in UBA1 Define a Distinct Subset of Relapsing Polychondritis Patients With VEXAS.
Ferrada MA, Sikora KA, Luo Y, Wells KV, Patel B, Groarke EM, Ospina Cardona D, Rominger E, Hoffmann P, Le MT, Deng Z, Quinn KA, Rose E, Tsai WL, Wigerblad G, Goodspeed W, Jones A, Wilson L, Schnappauf O, Laird RS, Kim J, Allen C, Sirajuddin A, Chen M, Gadina M, Calvo KR, Kaplan MJ, Colbert RA, Aksentijevich I, Young NS, Savic S, Kastner DL, Ombrello AK, Beck DB, Grayson PC. Ferrada MA, et al. Arthritis Rheumatol. 2021 Oct;73(10):1886-1895. doi: 10.1002/art.41743. Epub 2021 Aug 31. Arthritis Rheumatol. 2021. PMID: 33779074
OBJECTIVE: Somatic mutations in UBA1 cause a newly defined syndrome known as VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome). ...The random forest method was used to derive a clinical algorithm to identify patients with …
OBJECTIVE: Somatic mutations in UBA1 cause a newly defined syndrome known as VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammator …
Pulmonary manifestations in VEXAS syndrome.
Casal Moura M, Baqir M, Tandon YK, Samec MJ, Hines AS, Reichard KK, Mangaonkar AA, Go RS, Warrington KJ, Patnaik MM, Koster MJ, Ryu JH. Casal Moura M, et al. Respir Med. 2023 Jul;213:107245. doi: 10.1016/j.rmed.2023.107245. Epub 2023 Apr 14. Respir Med. 2023. PMID: 37062498
BACKGROUND: Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a recently recognized multisystem disorder caused by somatic mutations in the UBA1 gene. METHODS: A retrospective cohort study was conducted on all patients with VEXAS syndro …
BACKGROUND: Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a recently recognized multisystem dis …
Eye and Behçet's disease.
Ksiaa I, Abroug N, Kechida M, Zina S, Jelliti B, Khochtali S, Attia S, Khairallah M. Ksiaa I, et al. J Fr Ophtalmol. 2019 Apr;42(4):e133-e146. doi: 10.1016/j.jfo.2019.02.002. Epub 2019 Mar 5. J Fr Ophtalmol. 2019. PMID: 30850197 Review.
It is clinically characterized by oral and genital ulcerations, ocular inflammatory involvement, skin lesions, vascular involvement and numerous other systemic manifestations. ...
It is clinically characterized by oral and genital ulcerations, ocular inflammatory involvement, skin lesions, vascular inv …
Dermatology facing autoinflammatory syndrome.
Alecu M, Coman G, Muşetescu A, Cojoacă ME, Coman OA. Alecu M, et al. Rom J Morphol Embryol. 2015;56(1):7-14. Rom J Morphol Embryol. 2015. PMID: 25826481 Free article. Review.
Several skin disorders (pyoderma gangrenosum and pustular acne) may be encountered either isolate or associated with autoinflammatory symptoms, forming well-defined clinical entities within the autoinflammatory syndrome. ...Recently, dermatology …
Several skin disorders (pyoderma gangrenosum and pustular acne) may be encountered either isolate or associated with autoinflammat …
Autoinflammatory associated vasculitis.
Ginsberg S, Rosner I, Rozenbaum M, Slobodin G, Zilber K, Boulman N, Kaly L, Awisat A, Jiries N, Beyar-Katz O, Rimar D. Ginsberg S, et al. Semin Arthritis Rheum. 2016 Dec;46(3):367-371. doi: 10.1016/j.semarthrit.2016.07.007. Epub 2016 Jul 20. Semin Arthritis Rheum. 2016. PMID: 27612399
The skin is commonly involved with various manifestations including erysipelas like rash and urticaria. ...We suggest the term autoinflammatory associated vasculitis to describe vasculitis associated with autoinflammatory diseases. ...
The skin is commonly involved with various manifestations including erysipelas like rash and urticaria. ...We suggest the term …
Drug management of neutrophilic dermatoses.
Garcovich S, De Simone C, Berti E, Marzano AV. Garcovich S, et al. Expert Rev Clin Pharmacol. 2017 Oct;10(10):1119-1128. doi: 10.1080/17512433.2017.1356719. Epub 2017 Jul 27. Expert Rev Clin Pharmacol. 2017. PMID: 28715916 Review.
Clinical management is challenging, due to limited clinical evidence and lack of clinical practice guidelines. ...Another promising treatment strategy is aimed at blocking downstream effector cytokines, such as IL12/23 and IL-17, involved in the aut …
Clinical management is challenging, due to limited clinical evidence and lack of clinical practice guidelines. ...Anoth …
The Schnitzler syndrome.
Lipsker D. Lipsker D. Orphanet J Rare Dis. 2010 Dec 8;5:38. doi: 10.1186/1750-1172-5-38. Orphanet J Rare Dis. 2010. PMID: 21143856 Free PMC article. Review.
The Schnitzler syndrome is a rare and underdiagnosed entity which is considered today as being a paradigm of an acquired/late onset auto-inflammatory disease. ...Anakinra allows a complete control of all signs within hours after the first injection, but patients need conti …
The Schnitzler syndrome is a rare and underdiagnosed entity which is considered today as being a paradigm of an acquired/late onset a …
How to diagnose a lipodystrophy syndrome.
Vantyghem MC, Balavoine AS, Douillard C, Defrance F, Dieudonne L, Mouton F, Lemaire C, Bertrand-Escouflaire N, Bourdelle-Hego MF, Devemy F, Evrard A, Gheerbrand D, Girardot C, Gumuche S, Hober C, Topolinski H, Lamblin B, Mycinski B, Ryndak A, Karrouz W, Duvivier E, Merlen E, Cortet C, Weill J, Lacroix D, Wémeau JL. Vantyghem MC, et al. Ann Endocrinol (Paris). 2012 Jun;73(3):170-89. doi: 10.1016/j.ando.2012.04.010. Epub 2012 Jun 28. Ann Endocrinol (Paris). 2012. PMID: 22748602 Review.
The genetically determined partial forms usually occur as Dunnigan syndrome, which is a type of laminopathy that can also manifest as muscle, cardiac, neuropathic or progeroid involvement. ...Glycosylation disorders are sometimes involved. Some genetically de …
The genetically determined partial forms usually occur as Dunnigan syndrome, which is a type of laminopathy that can also manifest as …
47 results