Last update 21 Mar 2024
Schnitzler Syndrome
Last update 21 Mar 2024
Basic Info
Synonyms Chronic urticaria with gammopathy, Chronic urticaria with macroglobulinemia, Schnitzler Syndrome + [8] |
Introduction An extremely rare condition manifested as monoclonal IMMUNOGLOBULIN M dysproteinemia without features of lymphoproliferative disease, but with chronic urticaria, fever of unknown origin, disabling bone pain, hyperostosis, and increased erythrocyte sedimentation rate. |
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A Randomized, Double-Blind, Placebo-Controlled, Multicenter, Phase 2 Study of the Efficacy and Safety of RPH-104 in Adult Subjects With Schnitzler Syndrome
Development of an International Multicenter Registry of Patients With Monogenic and Polygenic Autoinflammatory Diseases Aimed at Clinical and Therapeutical Data Collection and Analysis
A Pilot, Open-Label, Phase 2, Single-Center, Repeat Dose, Proof- Of-Concept Safety, Pharmacodynamics and Efficacy Study of Orally Administered Dapansutrile Capsules in Subjects With Schnitzler's Syndrome
100 Clinical Results associated with Schnitzler Syndrome
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100 Translational Medicine associated with Schnitzler Syndrome
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08 Feb 2024Clinics in dermatology
Eponyms That Honor Jewish Dermatologists: A Celebration and a Remembrance Part Three: Jewish Physicians Who Practiced During the Holocaust and in its Aftermath.
Article
Author: By Leonard J Hoenig ; Dan Lipsker ; Lawrence Charles Parish
Part III of this contribution continues to celebrate the many contributions that Jewish physicians have made to advance the specialty of dermatology, as reflected by eponyms that honor their names. Part I covered the years before 1933, a highly productive period of creativity by Jewish dermatologists especially in Germany and Austria. The lives of 17 Jewish physicians and their eponyms were presented in Part I. Part II focused on the years from 1933 to 1945, when the Nazis rose to power in Europe and how their anti-Semitic genocidal policies impacted leading Jewish dermatologists caught within the Third Reich. Fourteen Jewish physicians and their eponyms were discussed in Part II. Part III continues the remembrance of the Holocaust era by looking at the careers and eponyms of an additional 13 Jewish physicians who contributed to dermatology during the period of 1933-1945. Two of these thirteen physicians, the pathologist Ludwig Pick (1868-1944) and the neurologist Arthur Simons (1877-1942) perished in the Holocaust. They are remembered by the following eponyms of interest to dermatologists: Lubarsch-Pick syndrome, Niemann-Pick disease and Barraquer-Simons syndrome. Four of the thirteen Jewish physicians escaped the Nazis: Felix Pinkus (1868-1947), Herman Pinkus (1905-1985), Arnault Tzanck (1886-1954) and Erich Urbach (1893-1946). Eponyms that honor their names include nitidus Pinkus, fibroepithelioma of Pinkus, Tzanck test, Urbach-Wiethe disease, Urbach-Koningstein technique, Oppenheim-Urbach disease, Extracellular cholesterinosis of Karl-Urbach. The other seven Jewish physicians lived outside the reach of the Nazis, either in Canada, the United States, or Israel. Their eponyms are discussed in this contribution. Part III also discusses eponyms that honor seven contemporary Jewish dermatologists who practiced dermatology after 1945 and who continue the nearly 200 years of Jewish contribution to the development of the specialty. They are A Bernard Ackerman (1936-2008), Irwin M. Braverman, Sarah Brenner, Israel Chanarin, Maurice L. Dorfman, Dan Lipsker, and Ronni Wolf. Their eponyms are Ackerman syndrome, Braverman sign, Brenner sign, Chanarin-Dorfman Syndrome, Lipsker criteria of the Schnitzler syndrome, and Wolf's isotopic response.
06 Dec 2023The Journal of dermatology
Neutrophilic epitheliotropism, proposed as an auto-inflammatory condition of neutrophilic urticarial dermatosis including Schnitzler syndrome, is also observed in Japanese cases.
Article
Author: Hitomi Nakaizumi ; Naotomo Kambe ; Hiroyuki Irie ; Yo Kaku ; Masakazu Fujimoto ; Hajime Yoshifuji ; Yasuhiro Kazuma ; Kazumoto Katagiri ; Takuro Kanekura ; Kenji Kabashima
Schnitzler syndrome (SchS) is a rare autoinflammatory disease characterized by bone pain, recurrent fever, leukocytosis, and elevated C-reactive protein, along with an urticaria-like rash and monoclonal immunoglobulin (Ig)M or IgG gammopathy. Notably, the condition is distinguished by a relatively persistent recurrent urticarial-like rash. Histopathological features observed in the skin comprise diffuse neutrophil infiltration into the dermis, absence of dermal edema, and vascular wall degeneration, all of which classify SchS as a neutrophilic urticarial dermatosis (NUD). Accumulated histological data from skin biopsies of patients with NUD have revealed a sensitive histopathological marker for NUD, acknowledged as neutrophilic epitheliotropism, which has been proposed as reflecting an autoinflammatory condition. In this report, we present three SchS patients: two men (ages 55 and 68) and a woman (age 75), all displaying neutrophilic epitheliotropism in their skin biopsy specimens. Additionally, a review of eight previously reported SchS cases in Japan identified neutrophilic epithliotropism in five cases. These findings suggest that the inclination of neutrophils toward the epithelial tissue could aid in confirming diagnoses of NUD in most cases that need to be differentiated from conventional urticaria. Consequently, we emphasize that acknowledging neutrophilic epithelial predilection as a hallmark of NUD is critical for expediting early diagnosis and appropriate treatment for SchS.
01 Dec 2023The Journal of dermatological treatment
Schnitzler syndrome refractory to anakinra: successful treatment with canakinumab.
Article
Author: Angel Luis Salcedo-Mingoarranz ; María Dorado-Fernández ; Sofía García-Martínez ; Paz Collado-Ramos ; Nicolás Silvestre-Torner
Schnitzler syndrome (SchS) is a rare autoimmune and inflammatory disease mediated by interleukin-1 beta (IL-1β). Recurrent monoclonal gammopathy and chronic urticarial rash are the symptoms required for diagnosis according to the Strasbourg criteria. The low prevalence of this syndrome (around 300 cases have been reported) and confusion with other inflammatory disorders may delay the diagnosis for up to 5 years. Although the most effective treatment for SchS is anakinra, some patients do not respond to this treatment. We report a case of SchS in a 64-year-old woman with multiple episodes of fever, severe rash, erythema, arthralgia and dyspnea. The patient was successfully treated with canakinumab after anakinra intolerance and failure of colchicine, prednisone, methotrexate and dapsone. After the first dose of canakinumab the skin wounds rapidly improved and the patient did not require any concomitant treatments. The cause of SchS is still unknown and a differential diagnosis is recommended, especially with adult-onset Still´s disease due to their similar symptoms. Canakinumab, a specific anti-IL-1β antibody, blocks its binding to receptors, thereby preventing IL-1β-induced gene activation and production of inflammatory mediators. Canakinumab has proven to be an effective drug in SchS, providing an alternative to anakinra.
11 Nov 2022
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