Research & Literature

Urticarial vasculitis and urticarial autoinflammatory syndromes.

Marzano AV, Tavecchio S, Venturini M, et al.

Giornale italiano di dermatologia e venereologia : organo ufficiale, Societa italiana di dermatologia e sifilografia 2015; (150(1)):41-50.

Correlative bone imaging in a case of Schnitzler's syndrome and brief review of the literature.

Willekens I, Walgraeve N, Goethals L, De Geeter F

Hellenic journal of nuclear medicine 2015; (18(1)):71-3.

Mast-cell interleukin-1β, neutrophil interleukin-17 and epidermal antimicrobial proteins in the neutrophilic urticarial dermatosis in Schnitzler's syndrome.

de Koning HD, van Vlijmen-Willems IM, Rodijk-Olthuis D, et al.

The British journal of dermatology 2015; (173(2)):448-56 doi:10.1111/bjd.13857.

The role of interleukin-1 beta in the pathophysiology of Schnitzler's syndrome.

de Koning HD, Schalkwijk J, Stoffels M, et al.

Arthritis research & therapy 2015; (17()):187 doi:10.1186/s13075-015-0696-0.

[Schnitzler syndrome is a diagnostic challenge].

Duhn PH, Thomsen SF, Nordin H

Ugeskrift for laeger 2015; (177(30)).

Neutrophilic Epitheliotropism is a Histopathological Clue to Neutrophilic Urticarial Dermatosis.

Broekaert SM, Böer-Auer A, Kerl K, et al.

The American Journal of dermatopathology 2016; (38(1)):39-49 doi:10.1097/DAD.0000000000000390.

Interleukin-1 receptor antagonist (anakinra) for Schnitzler syndrome.

Sönnichsen A, Saulite I, Mangana J, et al.

The Journal of dermatological treatment 2016; (27(5)):436-8 doi:10.3109/09546634.2015.1136048.

Schnitzler Syndrome: A Dramatic Response to Anakinra.

Gouveia AI, Micaelo M, Pierdomenico F, Freitas JP

Dermatology and therapy 2016; (6(2)):299-302 doi:10.1007/s13555-016-0108-7.

Clinical characterization and long-term follow-up of Schnitzler syndrome.

Gameiro A, Gouveia M, Pereira M, et al.

Clinical and experimental dermatology 2016; (41(5)):461-7 doi:10.1111/ced.12828.

Schnitzler Syndrome Without a Monoclonal Gammopathy: A Case Report.

Urbanski M, Holfeld K, Milne A, Abbas M

Journal of cutaneous medicine and surgery 2016; (20(6)):575-578 doi:10.1177/1203475416657999.

Schnitzler syndrome: validation and applicability of diagnostic criteria in real-life patients.

Gusdorf L, Asli B, Barbarot S, et al.

Allergy 2017; (72(2)):177-182 doi:10.1111/all.13035.

The Schnitzler syndrome: chronic urticaria in disguise: a single-centre report of 11 cases and a critical reappraisal of the literature.

Vanderschueren S, van der Veen A

Clinical and experimental rheumatology 2017; (35(1)):69-73.

Efficacy and safety of canakinumab in Schnitzler syndrome: A multicenter randomized placebo-controlled study.

Krause K, Tsianakas A, Wagner N, et al.

The Journal of allergy and clinical immunology 2017; (139(4)):1311-1320 doi:10.1016/j.jaci.2016.07.041.

[Schnitzlers SyndromeDifferential diagnostics, an overview of therapeutic options and description of 5 cases treated with anakinra].

Adam Z, Šedivá A, Koukalová R, et al.

Vnitrni lekarstvi 2016; (62(9)):713-727.

Schnitzler Syndrome: a Review.

Gusdorf L, Lipsker D

Current rheumatology reports 2017; (19(8)):46 doi:10.1007/s11926-017-0673-5.

Kidney involvement in the Schnitzler syndrome, a rare disease.

Basile C, Rossi L, Casucci F, et al.

Clinical kidney journal 2017; (10(6)):723-727 doi:10.1093/ckj/sfx077.

Molecular genetic investigation, clinical features, and response to treatment in 21 patients with Schnitzler syndrome.

Rowczenio DM, Pathak S, Arostegui JI, et al.

Blood 2018; (131(9)):974-981 doi:10.1182/blood-2017-10-810366.

A Rare but Fascinating Disorder: Case Collection of Patients with Schnitzler Syndrome.

Bashir M, Bettendorf B, Hariman R

Case reports in rheumatology 2018; (2018()):7041576 doi:10.1155/2018/7041576.

Neutrophilic urticarial dermatosis: A review.

Gusdorf L, Lipsker D

Annales de dermatologie et de venereologie 2018; (145(12)):735-740 doi:10.1016/j.annder.2018.06.010.

Association of CCL2 with systemic inflammation in Schnitzler syndrome.

Krause K, Sabat R, Witte-Händel E, et al.

The British journal of dermatology 2019; (180(4)):859-868 doi:10.1111/bjd.17334.

Different Therapeutic Paths (Colchicine vs. Anakinra) in Two Patients With Schnitzler's Syndrome.

Barešić M, Mitrović J, Morović Vergles J, Anić B

Archives of rheumatology 2016; (31(4)):377-380 doi:10.5606/ArchRheumatol.2016.6000.

Refractory urticaria and the importance of diagnosing Schnitzler's syndrome.

Nham T, Saleh C, Chu D, et al.

BMJ case reports 2019; (12(4)) doi:10.1136/bcr-2018-228546.

Diagnostic value of 18-F fluorodeoxyglucose PET/CT and bone scan in Schnitzler syndrome.

Alix L, Néel A, Cador B, et al.

Autoimmunity 2019; (52(7-8)):264-271 doi:10.1080/08916934.2019.1680649.

Efficacy and safety of canakinumab treatment in schnitzler syndrome: A systematic literature review.

Betrains A, Staels F, Vanderschueren S

Seminars in arthritis and rheumatism 2020; (50(4)):636-642 doi:10.1016/j.semarthrit.2020.05.002.

Rheumatic involvement and bone scan features in Schnitzler syndrome: initial and follow-up data from a single-center cohort of 25 patients.

Darrieutort-Laffite C, Ansquer C, Aubert H, et al.

Arthritis research & therapy 2020; (22(1)):272 doi:10.1186/s13075-020-02318-5.

Schnitzler Syndrome: the paradigm of an acquired adult-onset auto-inflammatory disease.

Gusdorf L, Lipsker D

Giornale italiano di dermatologia e venereologia : organo ufficiale, Societa italiana di dermatologia e sifilografia 2020; (155(5)):567-573 doi:10.23736/S0392-0488.20.06692-4.

A novel histopathological scoring system to distinguish urticarial vasculitis from chronic spontaneous urticaria.

Puhl V, Bonnekoh H, Scheffel J, et al.

Clinical and translational allergy 2021; (11(2)):e12031 doi:10.1002/clt2.12031.

Transformation of IgM-MGUS into Waldenström´s macroglobulinemia in two of six patients treated for Schnitzler´s syndrome.

Adam Z, Tomíška M, Řehák Z, et al.

Vnitrni lekarstvi 2021; (67(E-3)):15-23.

Schnitzler's Syndrome: A Diagnostic Consideration in Evaluating the Constellation of Monoclonal Gammopathy and Chronic Urticaria.

Mamadgi J, Babar L, Bhagavatula R, et al.

Journal of hematology 2021; (10(3)):143-146 doi:10.14740/jh800.

Refractory serum immunoglobulin M elevation during anti-interleukin (IL)-1- or IL-6-targeted treatment in four patients with Schnitzler syndrome.

Takimoto-Ito R, Kambe N, Kogame T, et al.

The Journal of dermatology 2021; (48(11)):1789-1792 doi:10.1111/1346-8138.16124.

Schnitzler syndrome and Schnitzler-like syndromes.

Chu CQ

Chinese medical journal 2022; (135(10)):1190-1202 doi:10.1097/CM9.0000000000002015.

A man in his fifties with recurrent urticaria, fever and joint pain.

Simonsen HE, Berkman R, Skorpen PK, et al.

Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke 2022; (142(4)) doi:10.4045/tidsskr.21.0028.

A patient with urticarial lesions, recurrent fever, and IgM-type monoclonal gammopathy.

Kecelj Žgank B, Benko M

Acta dermatovenerologica Alpina, Pannonica, et Adriatica 2022; (31(Suppl)):S27-S29.

Hypocomplementemic Urticarial Vasculitis Syndrome or Systemic Lupus Erythematosus in Evolution?

Kesarwani V, Phachu D, Trivedi R

Cureus 2022; (14(3)):e23429 doi:10.7759/cureus.23429.

Summary of the current status of clinically diagnosed cases of Schnitzler syndrome in Japan.

Takimoto-Ito R, Kambe N, Kogame T, et al.

Allergology international : official journal of the Japanese Society of Allergology 2023; (72(2)):297-305 doi:10.1016/j.alit.2022.11.004.

Refractory adult-onset Still's disease complicated with monoclonal gammopathy of undetermined significance: A case report.

Saito K, Asano T, Matsumoto H, et al.

Medicine 2022; (101(49)):e32107 doi:10.1097/MD.0000000000032107.

Similarities and differences in autoinflammatory diseases with urticarial rash, cryopyrin-associated periodic syndrome and Schnitzler syndrome.

Matsuda T, Takimoto-Ito R, Lipsker D, Kambe N

Allergology international : official journal of the Japanese Society of Allergology 2023; (72(3)):385-393 doi:10.1016/j.alit.2023.02.005.

Case report: Schnitzler-like syndrome without monoclonal gammopathy.

Wesselmann AS, Künstner A, Fähnrich A, et al.

Frontiers in immunology 2023; (14()):1166620 doi:10.3389/fimmu.2023.1166620.

Beyond monoclonal gammopathy of undetermined significance, clinical spectrum of immunoglobulin M gammopathy: a case series with focus on the diagnostic and management challenges.

Ashruf OS, Mirzai S, George LL, Anwer F

International journal of hematologic oncology 2023; (12(2)):IJH44 doi:10.2217/ijh-2022-0006.

Therapy resistant urticaria as a long-term symptom of an incomplete Schnitzler syndrome.

Puxkandl V, Currie A, Hoetzenecker W, Altrichter S

Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology 2023; (19(1)):64 doi:10.1186/s13223-023-00819-x.

Schnitzler syndrome refractory to anakinra: successful treatment with canakinumab.

Salcedo-Mingoarranz AL, Dorado-Fernández M, García-Martínez S, et al.

The Journal of dermatological treatment 2023; (34(1)):2242705 doi:10.1080/09546634.2023.2242705.

The many faces of pediatric urticaria.

Sekerel BE, Ilgun Gurel D, Sahiner UM, et al.

Frontiers in allergy 2023; (4()):1267663 doi:10.3389/falgy.2023.1267663.

Clinical characteristics, disease trajectories and management of vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome: a systematic review.

Kouranloo K, Dey M, Almutawa J, et al.

Rheumatology international 2024; (44(7)):1219-1232 doi:10.1007/s00296-023-05513-0.

Neutrophilic Urticarial Dermatosis.

Orakwue A, Bray J, Comfere N, Sokumbi O

Dermatologic clinics 2024; (42(2)):219-229 doi:10.1016/j.det.2023.08.009.

FACS-based detection of extracellular ASC specks from NLRP3 inflammasomes in inflammatory diseases.

Topping J, Lara-Reyna S, Ibbotson A, et al.

Clinical and experimental immunology 2025; (219(1)) doi:10.1093/cei/uxae117.

Background and Clinical Features of a Unique and Mysterious Autoinflammatory Disease, Schnitzler Syndrome.

Műzes G, Sipos F

International journal of molecular sciences 2025; (26(2)) doi:10.3390/ijms26020598.

Neutrophils predominate as IL1B-expressing cells in Schnitzler syndrome: Insights from the SCan study to evaluate the efficacy and safety of canakinumab in Japanese patients.

Kambe N, Inoue N, Ueki Y, et al.

Allergology international : official journal of the Japanese Society of Allergology 2025; (74(4)):605-615 doi:10.1016/j.alit.2025.04.003.

Schnitzler syndrome - a rare cause of chronic urticaria. Case report.

Moos Ł, Kułakowska A, Szydłowska D, et al.

Oxford medical case reports 2025; (2025(7)):omaf118 doi:10.1093/omcr/omaf118.

Clinical and laboratory markers to distinguish VEXAS from Schnitzler's syndrome: data from the AIDA network registries.

Caggiano V, Sbalchiero J, Frassi M, et al.

Frontiers in medicine 2025; (12()):1659758 doi:10.3389/fmed.2025.1659758.

Canakinumab as effective therapy in anakinra-refractory Schnitzler syndrome: a case-based review.

De Santos Belinchón S, Ausín García C, Lorenzo Barcia PD, et al.

Internal medicine journal 2026; (56(2)):306-310 doi:10.1111/imj.70233.

Long-term anakinra therapy in Schnitzler syndrome: real-world evidence on remission of neutrophilic dermatitis and systemic inflammation, safety, biomarker insights and dose optimization.

Sikora M, Ziętkiewicz M, Jahnz-Różyk K, Więsik-Szewczyk E

Frontiers in immunology 2026; (17()):1830866 doi:10.3389/fimmu.2026.1830866.

Club Rhumatismes et Inflammation guidelines for anti-interleukin-1 therapy: 2026 update.

Mitrovic S, Delplanque M, Ea HK, et al.

Joint bone spine 2026; 106084 doi:10.1016/j.jbspin.2026.106084.

Chronic Urticaria-Associated Syndromes: Is Andersen-Tawil Syndrome One of Them?

Bulat V, Zanze L, Peček M, et al.

Genes 2026; (17(8)) doi:10.3390/genes17080976.