Schnitzler Syndrome: Insights into Its Pathogenesis, Clinical Manifestations, and Current Management.

Schnitzler syndrome is a rare disorder characterized by a chronic urticarial rash associated with immunoglobulin M (IgM) monoclonal gammopathy. Schnitzler syndrome shares strong clinicopathologic similarities with monogenic IL-1-mediated autoinflammatory disorders and is now considered an acquired adult-onset autoinflammatory disease. The spectacular effect of interleukin-1 inhibitors demonstrates the key role of this cytokine in the pathogenesis of the disease. However, the physiopathology of Schnitzler syndrome remains elusive, and the main question regarding the relationship between autoinflammatory features and monoclonal gammopathy is still unanswered. The purpose of this narrative review is to describe what is currently known about the pathogenesis of this peculiar disease, as well as to address its diagnosis and management.

Eponyms that honor Jewish dermatologists: A celebration and a remembrance, Part three: Jewish physicians who practiced during the Holocaust and in its aftermath.

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 described in Part I. Part II focused on the years of 1933 to 1945, when the Nazis rose to power in Europe, and how their anti-Semitic genocidal policies affected leading Jewish dermatologists caught within the Third Reich. Fourteen Jewish physicians and their eponyms are 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 to 1945. Two of these 13 physicians, pathologist Ludwig Pick (1868-1944) and 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 13 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, and extracellular cholesterinosis of Karl-Urbach. The other seven Jewish physicians lived outside the reach of the Nazis, in either 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.

Neoadjuvant in Situ and Systemic Immunotherapy with Lymph Node Cryoablation in Resectable Stage III Melanoma Metastasis: a Proof-of-Concept Study.

PURPOSE: Complete lymph node dissection is the recommended treatment for clinically detectable lymph nodes in stage III melanoma. This surgery is associated with substantial morbidity. We hypothesize that combining percutaneous imaging-guided cryoablation of locoregional lymph nodes metastases with neoadjuvant in situ and systemic immunotherapy could allow disease control and evaluate the feasibility of this combination in this proof-of-concept study. METHODS: We enrolled 15 patients with stage IIIB/IIIC melanoma. Patients were treated as follows: a single 240 mg flat dose infusion of nivolumab on day 1, cryoablation under local anesthesia using CT on day 2, and a single intralesional injection of 10-20 mg of ipilimumab into the lymphadenopathy treated by cryotherapy on day 3. Five-eight weeks after this procedure, complete lymph node dissection was performed according to routine care. The primary outcome measure of this study was feasibility, measured as the number of failures (i.e., inability to complete the entire procedure). RESULTS: The procedure was carried out successfully in 15 out of 15 patients with an observed number of failures of 0. The Bayesian analysis showed an estimated failure rate of 4.2% [0.2-20.6]. Eight patients (53%) had adverse events secondary to either immunotherapy or cryotherapy. Grade 3/4 events occurred in three patients, but all resolved quickly and patients could proceed to surgery as scheduled. Eight patients (53%) had a pathological complete or near complete response. CONCLUSION: Combining percutaneous cryotherapy with in situ ipilimumab and systemic nivolumab for stage III resectable melanoma is feasible with tolerable toxicity.

Eponyms and clinical entities from the land of Israel.

Seven eponyms have been pioneered by dermatologists in Israel: Brenner's sign, Chanarin-Dorfman syndrome, granulated sweetener packet sign, isopathic phenomenon of Sagher, lanolin paradox, Nakar-Ingber disease, and Wolf's isotopic response. In addition, there are three id reactions described by Israeli dermatologists: leishmanid, pediculid, and scabid. There is also the acronym PEMPHIGUS, which stands for the causative reasons for pemphigus. We celebrate these eponyms and clinical entities, which reflect the impressive progress made by dermatologists in Israel during the past century who have helped to build an academic, vibrant, and dynamic specialty in the Holy Land.