Schnitzler syndrome is a very rare autoinflammatory disease that seems to arise from an IgM monoclonal gammopathy [1] [2] [3] [4]. The pathogenesis still remains incompletely understood, but the clinical presentation has been well-documented in the literature. The cardinal feature of patients suffering from Schnitzler syndrome is a chronic urticarial rash [1] [2] [3]. Urticaria is the presenting manifestation in many individuals and appears years before other notable symptoms and signs of Schnitzler syndrome [3] [4] [5] [6]:

• Fever - A chronic course of fever that possesses an intermittent pattern is seen in 75% of patients [1] [3] [6] [7]. Fever often accompanies the onset of urticaria, while some cases have been described in whom the rash appeared after fever [1] [2].
• Pain - Myalgia, arthralgia, and bone pain are frequent manifestations seen in this syndrome, together with fatigue [1] [3] [4].
• Peripheral neuropathy - From 7%-56% of individuals suffer from peripheral neuropathy according to different studies [1] [3].
• Night sweats, weight loss, hepatomegaly, and splenomegaly are less commonly encountered [1] [2] [7].

Lymphadenopathy is also an important sign, being present in more than 40% of cases [1]. The clinical course of Schnitzler syndrome is often described as several-years long before the diagnosis is made [1] [6]. It is important to mention that similarly to other monoclonal gammopathies, a significant risk exists toward the development of a lymphoproliferative disorder, such as IgM myeloma or marginal B-cell lymphoma [1] [6].

• Fever

  Fever often accompanies the onset of urticaria, while some cases have been described in whom the rash appeared after fever. Pain - Myalgia, arthralgia, and bone pain are frequent manifestations seen in this syndrome, together with fatigue. [symptoma.com]

  In approximately 90% of patients Relapsing arthralgias: Concurrent with fever; reported in 80% of patients Bone pain: Concurrent with fever; reported in 70% of patients Myalgias: Concurrent with fever Lymphadenopathy Hepatomegaly Splenomegaly Fatigue [emedicine.com]

  Clinical Commissioning Policy: Anakinra to treat periodic fevers and autoinflammatory disorders (all ages) (PDF). p. 18. Retrieved 11 July 2018. ^ de Koning HD, Bodar EJ, van der Meer JW, Simon A (December 2007). [en.wikipedia.org]

  It is characterized by the presentation of a monoclonal band, especially IgM, as well as fever, asthenia and arthralgia. [ncbi.nlm.nih.gov]

• Weight Loss

  We describe here a 48-year-old woman with a monoclonal IgM gammopathy and a 3-year history of chronic pruritic urticarial dermatosis, unexplained fevers, chronic polyarthritis, lymphadenopathy, leukocytosis, hepatomegaly, and weight loss. [jhu.pure.elsevier.com]

  Night sweats, weight loss, hepatomegaly, and splenomegaly are less commonly encountered. Lymphadenopathy is also an important sign, being present in more than 40% of cases. [symptoma.com]

  loss Angioedema: Very rare The urticarial rash is characterized as follows: Pale-rose, slightly elevated papules and plaques Individual lesions are 0.5-3 cm in diameter New lesions appear daily Lesions last 12-24 hours and then disappear without sequelae [emedicine.com]

• Plethora

  The circulating monoclonal antibody classes (IgG and IgA lambda antibodies) as well as the morphology of cutaneous lesions (hyperpigmentation, limb edema, thickening of the skin, hypertrichosis and plethora) differentiate POEMS syndrome from Schnitzler [onlinelibrary.wiley.com]

• Intravenous Administration

  Rituximab was initiated as a result of the accompanying lymphoplasmacytic lymphoma, with weekly intravenous administrations of 375 mg/m2 for six doses, but skin lesions did not improve. [ascopubs.org]

• Fishing

  Flow cytometry and fluorescence in situ hybridization (FISH) studies were unrevealing for a malignant population. No amyloid deposits were noted on congo-red staining. Serum IL-6 was elevated at 22 pg/ml. [omicsonline.org]

• Pharyngeal Edema

  We present the case of a 48-year-old man with 5 years of recurrent urticarial lesions, slight itching, as well as lip, face and occasional pharyngeal edema. The patient had also suffered from fever and right hip and left knee arthralgia. [ncbi.nlm.nih.gov]

• Eye Pain

  Pain. 61 Sadig RR...Fraser CL 30794479 2019 18 Neutrophilic urticarial dermatosis: A review. 61 Gusdorf L...Lipsker D 30224079 2018 19 [Diagnosis and treatment of Schnitzler syndrome]. 61 Zhang XR...Fang MY 30612413 2018 20 [Schnitzler syndrome]. 61 [malacards.org]

  Fraser, The First Case Report of Schnitzler Syndrome Presenting with Eye Pain, Ocular Immunology and Inflammation, 10.1080/09273948.2018.1563704, (1-3), (2019). F. F. Gellrich and C. [doi.org]

• Urticaria

  Her urticaria was triggered by cooler temperatures and an ice cube test for cold induced urticaria was positive. In spite of aggressive therapies this patient remains symptomatic. [doi.org]

  From Wikidata Jump to navigation Jump to search Human disease Schnitzler syndrome (disorder) Chronic urticaria with macroglobulinemia Chronic urticaria with gammopathy Chronic urticaria with gammapathy edit English Schnitzler syndrome Human disease Schnitzler [wikidata.org]

  Patients typically carry a diagnosis of urticaria; however, their cutaneous symptoms fail to respond to typical urticaria therapies and lack symptoms such as pruritus. [jddonline.com]

• Red Skin Lesion

  The frequency of urticaria ranges from daily to a few times a year, and the extent of skin involvement differs greatly. Patients describe red skin lesions covering the trunk and extremities, sparing head, neck, palms and soles. [doi.org]

• Bone Pain

  Often, the areas of hyperostosis coincide with areas of symptomatic bone pain, such as the iliac bone, tibia, femur, and vertebral column. See Workup for more detail. [emedicine.com]

  Schnitzler syndrome encompasses monoclonal gammopathy, urticaria, inflammation, recurrent fever, bone pain and arthralgia, with occasional lymphadenopathy and/or hepatosplenomegaly. [ncbi.nlm.nih.gov]

  Schnitzler syndrome - tense, generalized chronic urticaria, joint or bone pain, and monoclonal gammopathy of kappa type. [medical-dictionary.thefreedictionary.com]

  Main observations: A 43-year-old woman presented to an allergy and immunology clinic with a 7 year history of chronic urticaria, angioedema with anaphylaxis, monoclonal gammopathy and bone pain. [doi.org]

  Schnitzler syndrome or Schnitzler's syndrome is a rare disease characterised by onset around middle age of chronic hives (urticaria) and periodic fever, bone pain and joint pain (sometimes with joint inflammation ), weight loss, malaise, fatigue, swollen [en.wikipedia.org]

• Arthralgia

  It is characterized by the presentation of a monoclonal band, especially IgM, as well as fever, asthenia and arthralgia. [ncbi.nlm.nih.gov]

  Schnitzler syndrome is an autoinflammatory disease characterized by chronic, nonpruritic urticaria in association with recurrent fever, bone pain, arthralgia or arthritis, and a monoclonal gammopathy, most often of the immunoglobulin M (IgM) subtype. [emedicine.com]

  Schnitzler syndrome with cold-induced urticaria Abstract Background: Schnitzler syndrome encompasses monoclonal gammopathy, urticaria, inflammation, recurrent fever, bone pain and arthralgia, with occasional lymphadenopathy and/or hepatosplenomegaly. [doi.org]

  Presenting signs and symptoms are an urticarial rash, intermittent fevers, myalgia, arthralgia, bone pain, lymphadenopathy, and peripheral neuropathy. [symptoma.com]

  Additionally, patients with SS may see multiple providers for nonspecific complaints of fever, lymphadenopathy, arthralgias, and bone pain. [jddonline.com]

• Pierre Robin Syndrome

  Pfaundler （1872-1947） Austrian pediatrician and Gertrud Hurler（1889-1965） German pediatrician Pick ピック Pick's disease ピック病 Pierre Robin ピエール　ロバン Pierre Robin syndrome ピエール　ロバン症候群 Pirquet ピルケー Pirquet reaction ピルケー反応 Plummer プラマー Plummer's disease プラマー [jams.med.or.jp]

A high index of suspicion is necessary in order to make the diagnosis of Schnitzler syndrome [1]. A thorough history should be obtained first, during which the onset of symptoms (particularly the rash and relapses of fever), their progression, as well as severity must be covered. The physical examination can only confirm the nonspecific findings, which is why laboratory studies are the crucial step in the workup. A complete blood count (CBC) will show anemia and leukocytosis with neutrophilia in a significant number of patients, whereas basic inflammatory parameters will reveal elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) [1] [4]. The detection of an IgM kappa chain raises solid evidence toward Schnitzler syndrome (the presence of IgG is also reported but in very few patients), although their levels may be paradoxically low at the time of diagnosis [1] [2] [4] [6]. Skin biopsy and subsequent histopathological examination is an equally important diagnostic tool, showing a neutrophilic infiltrate in a significant portion of tested patients [7]. Bone marrow biopsy could be recommended in some cases.

• Lymphopenia

  Lymphopenia was present, but no plasmacytosis was noted. Flow cytometry and fluorescence in situ hybridization (FISH) studies were unrevealing for a malignant population. No amyloid deposits were noted on congo-red staining. [omicsonline.org]

• Fibrinoid Necrosis

  Urticarial vasculitis can also mimic Schnitzler syndrome; however, with urticarial vasculitis, skin biopsy should reveal features of true vasculitis with fibrinoid necrosis of small vessel walls, which should not be present in Schnitzler syndrome. [hindawi.com]

  For example, the picture published by Tanneberger et al and interpreted as vasculitis lack the typical features of the latter, as there is no fibrinoid necrosis of vessel wall [ 13 ]. [ncbi.nlm.nih.gov]

  Urticarial vasculitis should be excluded; even though vasculitis was reported in 20% of SchS cases, true fibrinoid necrosis of the vessel walls was absent in 5 re-examined cases, as indicated before. [doi.org]

  For example, the picture published by Tanneberger et al and interpreted as vasculitis lack the typical features of the latter, as there is no fibrinoid necrosis of vessel wall [13]. [ojrd.biomedcentral.com]

  Fibrinoid deposits were seen around dilated superficial blood vessels. However, fibrinoid necrosis, the hallmark of fully developed leukocytoclastic vasculitis, was not seen. Immunofluorescence studies were negative. [journals.lww.com]

This treatment controls the condition but does not cure it. [en.wikipedia.org]

The exact pathogenesis is still unclear and treatment remains a challenge. [ncbi.nlm.nih.gov]

Schnitzler syndrome, an autoimmune–autoinflammatory syndrome: Report of two new cases and review of the literature, May, 2011 IL1RA _HUMAN, UniProtKB TREATMENT The treatment remains a challenge. [flipper.diff.org]

Prognosis The life span in patients with Schnitzler syndrome has not been shown to differ much from the general population.[1] Careful follow-up is advised, however. [dictionnaire.sensagent.leparisien.fr]

Schnitzler syndrome has a favourable prognosis in term of mortality with a survival rate of 94% after 15 years. [flipper.diff.org]

Prognosis The overall prognosis depends on the development of lymphoproliferative complications such as lymphoma, IgM myeloma or Waldenström's disease (see these terms). [orpha.net]

Our objectives are to systematically review disease characteristics of Schnitzler syndrome and collect follow-up information to gain insight into treatment efficacy and long-term prognosis. [ncbi.nlm.nih.gov]

Schnitzler syndrome: beyond the case reports: review and follow-up of 94 patients with an emphasis on prognosis and treatment. [Review] Seminars in Arthritis and Rheumatism. 37(3):137-48, 2007 Dec. Almerigogna F. Giudizi MG. Cappelli F. Romagnani S. [dermnetnz.org]

The etiology of Schnitzler syndrome is unclear, but the rapid response to the interleukin-1 receptor inhibitor anakinra underlines the pivotal role which the proinflammatory cytokine interleukin-1 may play in the pathophysiology of this potentially autoinflammatory [ncbi.nlm.nih.gov]

Etiology Etiology remains unclear but the syndrome is probably an acquired auto-inflammatory disorder. It shares many features with genetically determined auto-inflammatory diseases. [orpha.net]

Although the etiology of the syndrome is unknown, current evidence suggests this is an autoinflammatory syndrome. Recognition of this syndrome is critical since it is highly responsive to anakinra. [dovepress.com]

Rheumatology, Dubrava University Hospital, Zagreb, Croatia Keywords: Anakinra, canakinumab, colchicine, glucocorticoids, Schnitzler’s syndrome Abstract Schnitzler’s syndrome is a rare autoinflammatory syndrome with unidentified mechanism of disease and etiology [archivesofrheumatology.org]

Summary Epidemiology Prevalence is unknown and about 150 cases have been reported, primarily in Europe. Clinical description There is a slight male predominance and mean age of disease onset is 51 years. Time to diagnosis often exceeds 5 years. [orpha.net]

AA amyloidosis has also been reported in people with Schnitzler syndrome. [1] The life span in patients with Schnitzler syndrome has not been shown to differ much from the general population. [6] Epidemiology [ edit ] It is a rare condition; as of September [en.wikipedia.org]

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It is not yet firmly established whether the monoclonal immunoglobulin component plays a part in the pathophysiology of the urticarial lesions. [ncbi.nlm.nih.gov]

Pathophysiology The pathophysiology of Schnitzler syndrome is largely unclear. [onlinelibrary.wiley.com]

Medical condition: Treatment and prevention of gout flares in patients with frequent flares and for whom NSAIDs and/or colchicine are contraindicated, not tolerated or ineffective Disease: Version SOC Term Classification Code Term Level 12.0 10018627 [clinicaltrialsregister.eu]

The mannitol reduces vitreous volume and could prevent iris strangulation. YAG laser iridotomy performed one day prior to PKP was shown to prevent UZS. Intraoperative Some steps are needed to be taken during surgery to prevent the occurrence of UZS. [eyewiki.aao.org]

We hope that early treatment may prevent development of complications of CAPS such as deafness and eye damage. [ucl.ac.uk]

It investigates links to lifestyle and presents research on how the suppression of proinflammatory pathways may provide opportunities for both prevention and treatment of chronic diseases. [books.google.com]

When given at the start of an attack, the effect is dramatic, as the attack is usually completely prevented. There is some evidence that tonsillectomy can cure this syndrome, especially in children with persistently enlarged tonsils. [amyloidosis.org.uk]

1. Jain T, Offord CP, Kyle RA, Dingli D. Schnitzler syndrome: an under-diagnosed clinical entity. Haematologica. 2013;98(10):1581-1585.
2. De Koning HD. Schnitzler’s syndrome: lessons from 281 cases. Clin Transl Allergy. 2014;4:41.
3. Lipsker D. The Schnitzler syndrome. Orphanet J Rare Dis. 2010;5:38.
4. Simon A, Asli B, Braun-Falco M, et al. Schnitzler's syndrome: diagnosis, treatment, and follow-up. Allergy. 2013;68(5):562-568.
5. Gouveia AI, Micaelo M, Pierdomenico F, Freitas JP. Schnitzler Syndrome: A Dramatic Response to Anakinra. Dermatol Ther (Heidelb). 2016;6(2):299-302.
6. Kurian A, Lee JK, Vadas P. Schnitzler syndrome with cold-induced urticaria. J Dermatol Case Rep. 2010;4(4):50-53.
7. Sokumbi O, Drage LA, Peters MS. Clinical and histopathologic review of Schnitzler syndrome: the Mayo Clinic experience (1972-2011). J Am Acad Dermatol. 2012;67(6):1289-1295.