eMedicine Specialties > Dermatology > Allergy & Immunology

Schnitzler Syndrome: Follow-up

Author: Joel G DeKoven, MD, FRCPC, Associate Professor, Division of Dermatology, Department of Medicine, University of Toronto, Sunnybrook Health Sciences Centre and St Michael's Hospital, Canada
Coauthor(s): Kucy Pon, MD, FRCPC, Assistant Professor, Division of Dermatology, Department of Medicine, University of Toronto Faculty of Medicine
Contributor Information and Disclosures

Updated: Jun 30, 2009

Follow-up

Further Outpatient Care

Schnitzler syndrome requires long-term follow-up because of the potential for the development of lymphoproliferative disorders, especially Waldenström macroglobulinemia. Monitoring patients with periodic serum protein electrophoresis and reevaluation for lymphadenopathy and bone marrow involvement, if clinically indicated, is important.

Complications

With Schnitzler syndrome, a lymphoplasmacytic malignancy, such as Waldenström macroglobulinemia, lymphoplasmacytic lymphoma, or IgM myeloma, may occur.

Prognosis

No spontaneous complete remissions have been reported. Overall, the prognosis for Schnitzler syndrome is good. However, approximately 10-15% of patients develop a lymphoplasmacytic malignancy.

 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous Chief Editor, William D. James, MD, to the development and writing of this article.



More on Schnitzler Syndrome

Overview: Schnitzler Syndrome
Differential Diagnoses & Workup: Schnitzler Syndrome
Treatment & Medication: Schnitzler Syndrome
Follow-up: Schnitzler Syndrome
References
[ CLOSE WINDOW ]

References

  1. Schnitzler L, Schubert B, Boasson M. Urticaire chronique, lons osseuses, macroglobuline IgM: maladie de Waldenstrom. Bull Soc Franc Derm Syph. 1974;81:363.

  2. de Koning HD, Bodar EJ, van der Meer JW, Simon A,. Schnitzler syndrome: beyond the case reports: review and follow-up of 94 patients with an emphasis on prognosis and treatment. Semin Arthritis Rheum. Dec 2007;37(3):137-48. [Medline].

  3. Asli B, Bievenu B, Cardoliani F, et al. Chronic Urticaria and Monoclonal IgM gammopathy (Schnitzler Syndrome). Report of 11 cases treated with Pefloxacin. Arch Dermatol. 2007;143:1046-1050.

  4. Lipsker D, Veran Y, Grunenberger F, Cribier B, Heid E, Grosshans E. The Schnitzler syndrome. Four new cases and review of the literature. Medicine (Baltimore). Jan 2001;80(1):37-44. [Medline].

  5. Olsen E, Forre O, Lea T, Langeland T. Unique antigenic determinants (idiotypes) used as markers in a patient with macroglobulinemia and urticaria. Similar idiotypes demonstrated in the skin and on peripheral blood lymphocytes. Acta Med Scand. 1980;207(5):379-84. [Medline].

  6. Saurat JH, Schifferli J, Steiger G, Dayer JM, Didierjean L. Anti-interleukin-1 alpha autoantibodies in humans: characterization, isotype distribution, and receptor-binding inhibition--higher frequency in Schnitzler's syndrome (urticaria and macroglobulinemia). J Allergy Clin Immunol. Aug 1991;88(2):244-56. [Medline].

  7. Morita A, Sakakibara S, Yokota M, Tsuji T. A case of urticarial vasculitis associated with macroglobulinemia (Schnitzler's syndrome). J Dermatol. Jan 1995;22(1):32-5. [Medline].

  8. Simon A, van der Meer JWM, Drenth JPH. Familial auto-inflammatory syndromes. In: Harris ED, Budd RC, Firestein GS. Kelley's textbook of Rheumatology. 7th. Philadelphia: Saunders; 2004:Chapter 112 pp1773-88.

  9. Akimoto R, Yoshida M, Matsuda R, Miyasaka K, Itoh M. Schnitzler's syndrome with IgG kappa gammopathy. J Dermatol. Nov 2002;29(11):735-8. [Medline].

  10. de Koning HD, Bodar EJ, Simon A, van der Hilst JC, Netea MG, van der Meer JW. Beneficial response to anakinra and thalidomide in Schnitzler's syndrome. Ann Rheum Dis. Apr 2006;65(4):542-4. [Medline].

  11. Worm M, Kolde G. Schnitzler's syndrome: successful treatment of two patients using thalidomide. Br J Dermatol. Mar 2003;148(3):601-2. [Medline].

  12. Ramadan KM, Eswedi HA, El-Agnaf MR. Schnitzler syndrome: A case report of successful treatment using the anti-CD20 momoclonal antibody rituximab. Br J Dermatol. 2007;156:1072-74.

  13. Eiling E, Moller M, Kreiselmaier I, Brasch J, Schwarz T. Schnitzler syndrome: treatment failure to rituximab but response to anakinra. J Am Acad Dermatol. Aug 2007;57(2):361-4. [Medline].

  14. Martinez-Taboada VM, Fontalba A, Blanco R, Fernandez-Luna JL. Successful treatment of refractory Schnitzler syndrome with anakinra: comment on the article by Hawkins et al. Arthritis Rheum. Jul 2005;52(7):2226-7. [Medline].

  15. Bonnetblanc JM, Drouet M, Laplaud P, Bedane C, Bernard P. Urticaria with macroglobulinaemia. Disease activity associated alterations in immunoglobulins profile and bone marrow hypodiploidy. Dermatologica. 1990;181(1):41-3. [Medline].

  16. Borradori L, Rybojad M, Puissant A, Dallot A, Verola O, Morel P. Urticarial vasculitis associated with a monoclonal IgM gammopathy: Schnitzler's syndrome. Br J Dermatol. Jul 1990;123(1):113-8. [Medline].

  17. Harati A, Brockmeyer NH, Altmeyer P, Kreuter A. Skin disorders in association with monoclonal gammopathies. Eur J Med Res. Mar 29 2005;10(3):93-104. [Medline].

  18. Machet L, Vaillant L, Machet MC, Reisenleiter M, Goupille P, Lorette G. Schnitzler's syndrome (urticaria and macroglobulinemia): evolution to Waldenström's disease is not uncommon. Acta Derm Venereol. Sep 1996;76(5):413. [Medline].

  19. Nashan D, Sunderkotter C, Bonsmann G, Luger T, Goerdt S. Chronic urticaria, arthralgia, raised erythrocyte sedimentation rate and IgG paraproteinaemia: a variant of Schnitzler's syndrome?. Br J Dermatol. Jul 1995;133(1):132-4. [Medline].

  20. Puddu P, Cianchini G, Girardelli CR, Colonna L, Gatti S, de Pita O. Schnitzler's syndrome: report of a new case and a review of the literature. Clin Exp Rheumatol. Jan-Feb 1997;15(1):91-5. [Medline].

  21. Sanmartín O, Febrer I, Botella R, Grau M, de la Cuadra J, Aliaga A. Urticarial lesions and monoclonal IgM gammopathy. Schnitzler's syndrome. Arch Dermatol. Sep 1994;130(9):1195, 1198. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

Schnitzler syndrome, Schnitzler's syndrome, chronic urticaria, nonpruritic urticaria, monoclonal immunoglobulin M gammopathy, monoclonal IgM gammopathy

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Joel G DeKoven, MD, FRCPC, Associate Professor, Division of Dermatology, Department of Medicine, University of Toronto, Sunnybrook Health Sciences Centre and St Michael's Hospital, Canada
Joel G DeKoven, MD, FRCPC is a member of the following medical societies: American Academy of Dermatology, American Contact Dermatitis Society, Canadian Medical Association, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Coauthor(s)

Kucy Pon, MD, FRCPC, Assistant Professor, Division of Dermatology, Department of Medicine, University of Toronto Faculty of Medicine
Kucy Pon, MD, FRCPC is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for Laser Medicine and Surgery, Canadian Medical Association, and Canadian Medical Protective Association
Disclosure: Nothing to disclose.

Medical Editor

Jean-Hilaire Saurat, MD, Chair, Professor, Department of Dermatology, University of Geneva, Switzerland
Jean-Hilaire Saurat, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey Meffert, MD, Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-San Antonio
Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society
Disclosure: Nothing to disclose.

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

RELATED MEDSCAPE ARTICLES
Articles
 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.