R. Kyle, T. Therneau, S. Rajkumar, E. Remstein, J. Offord et al., Long-term follow-up of IgM monoclonal gammopathy of undetermined significance, Blood, vol.102, issue.10, pp.3759-64, 2003.
DOI : 10.1182/blood-2003-03-0801

K. Claes, B. Bammens, M. Delforge, P. Evenepoel, D. Kuypers et al., Another devastating complication of the Schnitzler syndrome: AA amyloidosis, British Journal of Dermatology, vol.193, issue.0, pp.182-186, 2008.
DOI : 10.1016/j.clim.2005.10.013

H. De-koning, E. Bodar, J. Van-der-meer, and A. Simon, Schnitzler Syndrome: Beyond the Case Reports: Review and Follow-Up of 94 Patients with an Emphasis on Prognosis and Treatment, Seminars in Arthritis and Rheumatism, vol.37, issue.3, pp.137-185, 2007.
DOI : 10.1016/j.semarthrit.2007.04.001

L. Schnitzler, Lésions urticariennes chroniques permanentes (érythème pétaloïde ?) Cas cliniques,n °4 6B .Journée Dermatologique d'Angers, 1972.

L. Schnitzler, B. Schubert, M. Boasson, J. Gardais, and A. Tourmen, Urticaire chronique, lésions osseuses, macroglobulinémie IgM: maladie de Waldenström ? 2 ème présentation, Bull Soc Fr Dermatol Syphil, vol.81, p.363, 1974.

D. Lipsker, Y. Veran, F. Grunenberger, B. Cribier, E. Heid et al., The Schnitzler Syndrome, Medicine, vol.80, issue.1, pp.37-44, 2001.
DOI : 10.1097/00005792-200101000-00004

URL : https://hal.archives-ouvertes.fr/inserm-00663708

H. Lachmann and P. Hawkins, Developments in the scientific and clinical understanding of autoinflammatory disorders, Arthritis Research & Therapy, vol.11, issue.1, p.212, 2009.
DOI : 10.1186/ar2579

M. Soubrier, J. Dubost, P. Jouanel, A. Tridon, B. Flori et al., Multiples complications d'une IgM monoclonale, La Revue de M??decine Interne, vol.15, issue.7, pp.484-490, 1994.
DOI : 10.1016/S0248-8663(05)81474-2

C. Kieffer, B. Cribier, and D. Lipsker, Neutrophilic Urticarial Dermatosis, Medicine, vol.88, issue.1, pp.23-31, 2009.
DOI : 10.1097/MD.0b013e3181943f5e

URL : http://doi.org/10.1097/md.0b013e3181943f5e

J. Clauvel, J. Brouet, F. Danon, M. Leibowitch, and M. Seligmann, Chronic urticaria with monoclonal IgM???A report of five cases, Clinical Immunology and Immunopathology, vol.25, issue.3, pp.348-53, 1982.
DOI : 10.1016/0090-1229(82)90199-4

G. Sánchez, M. Añó, C. García-avilés, and I. Diéguez, Schnitzler syndrome: a case study, J Investig Allergol Clin Immunol, vol.10, issue.1, pp.41-44, 2000.

F. De-castro, I. Masouyé, R. Winkelmann, and J. Saurat, Urticarial pathology in Schnitzler's (hyper-IgM) syndrome. Dermatology, pp.94-103, 1996.

O. Tanneberger, S. Buechner, and L. Zimmerli, Schnitzler-Syndrome mit Urtikaria-Vaskulitis, Internist (Berl), vol.2007, issue.4812, pp.1432-1437

P. Altmeyer and S. Welke, Makroglobulinämie Waldenström, assoziiert mit einem chronisch rezidivierenden urtikariellen Exanthem, Akt Dermatol, vol.3, pp.71-77, 1977.

S. Berdy and K. Bloch, Schnitzler's syndrome: A broader clinical spectrum, Journal of Allergy and Clinical Immunology, vol.87, issue.4, pp.849-54, 1991.
DOI : 10.1016/0091-6749(91)90132-8

L. Borradori, M. Rybojad, A. Puissant, A. Dallot, O. Verola et al., Urticarial vasculitis associated with a monoclonal IgM gammopathy: Schnitzler's syndrome, British Journal of Dermatology, vol.39, issue.1, pp.113-121, 1990.
DOI : 10.1016/0090-1229(82)90199-4

R. Carlioz, C. Haas, F. Jaubert, C. Allouche, W. Lowenstein et al., Maladie de Waldenström avec lymphome diffus à grandes cellules immunoblastiques et urticaire chronique, Ann Med Interne, vol.140, pp.51-78, 1989.

A. Morita, S. Sakakibara, M. Yokota, and T. Tsuji, A Case of Urticarial Vasculitis Associated with Macroglobulinemia (Schnitzler's Syndrome), The Journal of Dermatology, vol.20, issue.1, pp.32-37, 1995.
DOI : 10.1111/j.1346-8138.1995.tb03337.x

M. Janier, D. Bonvalet, M. Blanc, F. Lemarchand, B. Cavelier et al., Chronic urticaria and macroglobulinemia (Schnitzler's syndrome): Report of two cases, Journal of the American Academy of Dermatology, vol.20, issue.2, pp.206-217, 1989.
DOI : 10.1016/S0190-9622(89)70023-2

E. Olsen, O. Forre, T. Lea, and T. Langeland, Unique Antigenic Determinants (Idiotypes) Used as Markers in a Patient with Macroglobulinemia and Urticaria, Acta Medica Scandinavica, vol.114, issue.4, pp.379-84, 1980.
DOI : 10.1111/j.0954-6820.1980.tb09742.x

P. Puddu, G. Cianchini, C. Girardelli, C. L. Gatti, S. et al., Schnitzler's syndrome: report of a new case and review of the literature, Clin Exp Rheumatol, vol.15, pp.91-96, 1997.

D. Lipsker, D. Spehner, R. Drillien, P. Schmitt, B. Cribier et al., Schnitzler syndrome: heterogeneous immunopathologicalfindings involving IgM-skin interactions, British Journal of Dermatology, vol.30, issue.Suppl., pp.954-963, 2000.
DOI : 10.1046/j.1365-2133.2000.03477.x

E. Besada and H. Nossent, Dramatic response to IL1-RA treatment in longstanding multidrug resistant Schnitzler???s syndrome: a case report and literature review, Clinical Rheumatology, vol.27, issue.9, pp.567-71, 2010.
DOI : 10.1007/s10067-010-1375-9

M. Lecompte, G. Blais, G. Bisson, and B. Maynard, Schnitzler's syndrome, Skeletal Radiology, vol.27, issue.5, pp.294-300, 1998.
DOI : 10.1007/s002560050385

S. De-waele, F. Lecouvet, and J. Malghem, Schnitzler's Syndrome, American Journal of Roentgenology, vol.175, issue.5, pp.1325-1327, 2000.
DOI : 10.2214/ajr.175.5.1751325

F. Ferrando, J. Pujol, J. Hortells, M. Navarro, J. Pinol et al., Schnitzler's syndrome: report of a case with bone osteolysis, J Invest Allergol Clin Immunol, vol.4, pp.203-208, 1994.

D. Lambert, A. Legoux, J. Chapuis, J. Benoit, and R. Putelat, Urticaire chronique et macroglobulinémie, Nouv Presse Med, vol.11, 1948.

P. Germain, J. Fach, N. Bui, T. Traissac, X. Delbrel et al., Syndrome de Schnitzler: une cause rare d'urticaire syst??mique, La Revue de M??decine Interne, vol.21, issue.3, pp.285-294, 2000.
DOI : 10.1016/S0248-8663(00)80049-1

G. Winckelmann, H. Nagel, R. Maier, and G. Reuther, Schnitzler-Syndrom als Ursache eines rezidivierenden Fiebers unbekannter Ursache, DMW - Deutsche Medizinische Wochenschrift, vol.121, issue.27, pp.860-864, 1996.
DOI : 10.1055/s-2008-1043079

A. Flórez, E. Gallardo-agromayor, R. García-barredo, L. Alvaro, R. et al., Radiological aid to clinical diagnosis of Schnitzler???s syndrome: multimodality imaging approach, Clinical Rheumatology, vol.220, issue.7, pp.107-117, 2008.
DOI : 10.1007/s10067-007-0758-z

K. Chanchairujira, C. Chung, and Y. Lai, Intramedullary Osteosclerosis: Imaging Features in Nine Patients, Radiology, vol.220, issue.1, pp.225-230, 2001.
DOI : 10.1148/radiology.220.1.r01jl24225

M. Doutre, C. Beylot, P. Bioulac, and J. Bezian, Monoclonal IgM and chronic urticaria: two cases, Ann Allergy, vol.58, pp.413-417, 1987.

B. Welsh and B. Tate, Schnitzler???s syndrome: Report of a case with progression to Waldenstr??m???s macroglobulinaemia, Australasian Journal of Dermatology, vol.40, issue.4, pp.201-204, 1999.
DOI : 10.1046/j.1440-0960.1999.00361.x

H. Barrière, L. Schnitzler, G. Moulin, and Y. Grolleau, Lésions urticariennes chroniques et macroglobulinémie. A propos de cinq observations. Sem Hôp Paris, pp.221-228, 1976.

J. Clauvel, J. Brouet, F. Danon, M. Leibowitch, and M. Seligmann, Chronic urticaria with monoclonal IgM???A report of five cases, Clinical Immunology and Immunopathology, vol.25, issue.3, pp.348-53, 1982.
DOI : 10.1016/0090-1229(82)90199-4

P. Goupille, P. Pizzuti, E. Diot, F. Jattiot, J. Guilmot et al., Schnitzler's syndrome (urticaria with macroglobulinemia) dramatically improved with corticosteroids, Clin Exp Rheumatol, vol.13, pp.95-103, 1995.

L. Machet, L. Vaillant, M. Machet, E. Esteve, D. Muret et al., Schnitzler's syndrome (urticaria and macroglobulinemia) associated with pseudoxanthoma elasticum, Acta Derm Venereol, vol.72, pp.22-26, 1991.

P. Paulin, C. Cusset, J. L. Pages, J. Chapalain, and J. , Syndrome de Schnitzler: une nouvelle observation, Presse Med, vol.21, pp.1758-61, 1992.

E. Romanini, M. Magaletti, D. Aviera, L. Antonelli, and S. , Severe heterotopic ossification after total hip arthroplasty in Schnitzler syndrome, Hip Int, vol.20, pp.284-290, 2010.

D. Nashan, C. Sunderkötter, G. Bonsmann, T. Luger, and S. Goerdt, Chronic urticaria, arthralgia, raised erythrocyte sedimentation rate and IgG paraproteinaemia: a variant of Schnitzler's syndrome?, British Journal of Dermatology, vol.93, issue.1, pp.132-136, 1995.
DOI : 10.1111/j.1365-2133.1995.tb02507.x

R. Treudler, F. Kauer, and J. Simon, Striking effect of the IL-1 receptor antagonist Anakinra in chronic urticarial rash with polyclonal increase in IgA and IgG, Acta Derm Venereol, vol.87, pp.280-281, 2007.

M. Rybojad, I. Moraillon, F. Cordoliani, C. Lebbé, M. Baccard et al., Syndrome de Schnitzler aved déficit génétique en C4: deux observations, Ann Dermatol Venereol, vol.120, pp.783-788, 1993.

H. Tomkova, Y. Shirafuji, and J. Arata, Schnitzler's syndrome versus adult onset Still's disease, Eur J Dermatol, vol.8, pp.118-139, 1998.

L. Machet, L. Vaillant, M. Machet, E. Esteve, D. Muret et al., Schnitzler's syndrome (urticaria and macroglobulinemia) associated with pseudoxanthoma elasticum, Acta Derm Venereol, vol.72, pp.22-26, 1991.

M. Wilhelmi, O. Wilhelmi, J. , O. Sierra, and J. , Schnitzler's syndrome and pseudoxanthoma elasticum, p.159, 1993.

C. Lebbe, M. Rybojad, F. Klein, E. Oksenhendler, M. Catala et al., Schnitzler's syndrome associated with sensorimotor neuropathy, Journal of the American Academy of Dermatology, vol.30, issue.2, pp.316-324, 1994.
DOI : 10.1016/S0190-9622(94)70031-1

A. Lauwers, V. Chouvy, J. Mosnier, L. Misery, and C. Alexandre, A case of Schnitzler's syndrome with nodular regenerative hyperplasia of the liver, Rev Rhum Engl Ed, vol.66, pp.281-284, 1999.

T. Westhoff, W. Zidek, L. Uharek, J. Steinhoff-georgieva, and M. Van-der-giet, Impairment of renal function in Schnitzler's syndrome, J Nephrol, vol.19, pp.660-663, 2006.

G. Famularo, A. Barracchini, and G. Minisola, Severe thrombophilia with antiphospholipid syndrome and hyperhomocysteinemia in a patient with Schnitzler's syndrome, Clin Exp Rheumatol, vol.21, issue.3, pp.366-374, 2003.

P. Altmeyer and S. Welke, Makroglobulinämie Waldenström, assoziiert mit einem chronisch rezidivierenden urtikariellen Exanthem, Akt Dermatol, vol.3, pp.71-77, 1977.

S. Govindaraju, P. Brochot, A. Ringot, L. Paternotte, A. Morrone et al., Urticaire chronique-macroglobulin??mie (syndrome de Schnitzler): ??volution vers un my??lome ?? IgM. ?? propos d'un cas, La Revue de M??decine Interne, vol.14, issue.8, pp.780-783, 1993.
DOI : 10.1016/S0248-8663(05)81424-9

L. Machet, L. Vaillant, M. Machet, M. Reisenleiter, P. Goupille et al., Schnitzler's syndrome (urticaria and macroglobulinemia): evolution to Waldenström's disease is not uncommon, Acta Derm Venereol, vol.76, p.413, 1996.

R. Pujol, M. Barnadas, S. Brunet, and J. De-moragas, Urticarial dermatosis associated with Waldenstrom's macroglobulinemia, Journal of the American Academy of Dermatology, vol.20, issue.5, pp.855-862, 1989.
DOI : 10.1016/S0190-9622(89)80127-6

B. Welsh and B. Tate, Schnitzler???s syndrome: Report of a case with progression to Waldenstr??m???s macroglobulinaemia, Australasian Journal of Dermatology, vol.40, issue.4, pp.201-204, 1999.
DOI : 10.1046/j.1440-0960.1999.00361.x

S. Dalle, B. Balme, C. Sebban, C. Pariset, F. Berger et al., Schnitzler syndrome associated with systemic marginal zone B-cell lymphoma, British Journal of Dermatology, vol.133, issue.4
DOI : 10.1097/00005792-200101000-00004

M. Gilson, S. Abad, C. Larroche, and R. Dhote, Treatment of Schnitzler syndrome with anakinra, Clin Exp Rheumatol, vol.25, p.931, 2007.

J. Saurat, J. Schifferli, G. Steiger, J. Dayer, and L. Didierjean, Anti-interleukin-1?? autoantibodies in humans: Characterization, isotype distribution, and receptor-binding inhibition???Higher frequency in Schnitzler's syndrome (urticaria and macroglobulinemia), Journal of Allergy and Clinical Immunology, vol.88, issue.2, pp.244-56, 1991.
DOI : 10.1016/0091-6749(91)90335-L

H. Murota, Y. Shoda, T. Ishibashi, H. Sugahara, I. Matsumura et al., Improvement of recurrent urticaria in a patient with Schnitzler syndrome associated with B-cell lymphoma with combination rituximab and radiotherapy, Journal of the American Academy of Dermatology, vol.61, issue.6, pp.611070-611075, 2009.
DOI : 10.1016/j.jaad.2008.12.040

J. Ryan, H. De-koning, L. Beck, M. Booty, D. Kastner et al., IL-1 blockade in Schnitzler syndrome: Ex vivo findings correlate with clinical remission, Journal of Allergy and Clinical Immunology, vol.121, issue.1, pp.260-262, 2008.
DOI : 10.1016/j.jaci.2007.09.021

C. Pizzirani, S. Falzoni, M. Govoni, L. Corte, R. Donadei et al., Dysfunctional inflammasome in Schnitzler's syndrome, Rheumatology, vol.48, issue.10, pp.481304-481312, 2009.
DOI : 10.1093/rheumatology/kep222

P. Migliorini, D. Corso, I. Tommasi, C. Boraschi, and D. , Free circulating interleukin-18 is increased in the Schnitzler syndrome: a new autoinflammatory disease, Eur Cytokine Netw, vol.20, pp.108-119, 2009.

J. Loock, P. Lamprecht, C. Timmann, U. Mrowietz, E. Csernok et al., Genetic predisposition (NLRP3 V198M mutation) for IL-1???mediated inflammation in a patient with Schnitzler syndrome, Journal of Allergy and Clinical Immunology, vol.125, issue.2, pp.500-502, 2010.
DOI : 10.1016/j.jaci.2009.10.066

C. Schuster, B. Kränke, E. Aberer, E. Arbab, G. Sturm et al., Schnitzler syndrome: response to anakinra in two cases and a review of the literature, International Journal of Dermatology, vol.119, issue.11, pp.1190-1194, 2009.
DOI : 10.1111/j.1365-4632.2009.04151.x

B. Asli, B. Bienvenu, F. Cordoliani, J. Brouet, Y. Uzunhan et al., Chronic Urticaria and Monoclonal IgM Gammopathy (Schnitzler Syndrome), Archives of Dermatology, vol.143, issue.8, pp.1431046-50, 2007.
DOI : 10.1001/archderm.143.8.1046

E. Kastritis, A. Katoulis, E. Terpos, I. Panayiotides, M. Gavriatopoulopu et al., Schnitzler's Syndrome: Increased Levels of Bone Formation and Angiogenesis Factors Are Reduced After Successful Pefloxacin Treatment, Clinical Lymphoma and Myeloma, vol.8, issue.6, pp.359-62, 2008.
DOI : 10.3816/CLM.2008.n.053

V. Martinez-taboada, A. Fontalba, R. Blanco, and J. Fernández-luna, Successful treatment of refractory Schnitzler syndrome with anakinra: Comment on the article by Hawkins et al, Arthritis & Rheumatism, vol.364, issue.7, pp.2226-2233, 2005.
DOI : 10.1002/art.21101

N. Cascavilla, M. Bisceglia, D. Arena, and G. , Successful Treatment of Schnitzler's Syndrome with Anakinra after Failure of Rituximab Trial, International Journal of Immunopathology and Pharmacology, vol.23, issue.2, pp.633-639, 2010.
DOI : 10.1177/039463201002300226

P. Frischmeyer-guerrerio, R. Rachamalla, and S. Saini, REMISSION OF SCHNITZLER SYNDROME AFTER TREATMENT WITH ANAKINRA, Annals of Allergy, Asthma & Immunology, vol.100, issue.6, pp.617-626, 2008.
DOI : 10.1016/S1081-1206(10)60064-6

S. Masters, A. Simon, I. Aksentijevich, and D. Kastner, : The Molecular Pathophysiology of Autoinflammatory Disease, Annual Review of Immunology, vol.27, issue.1, pp.621-68, 2009.
DOI : 10.1146/annurev.immunol.25.022106.141627