Schnitzler Syndrome Associated With Multiple Myeloma: A Case Report

Abstract

Schnitzler syndrome is an autoimmune disease characterized with chronic urticarial rashes, monoclonal gammopathy, intermittent fever, arthralgia, arthritis, bone pain, lymphadenopathy, hepatosplenomegaly, leukocytosis and elevated erythrocyte sedimentation rate (ESR). Schnitzler syndrome must be investigated in urticaria which is long acting and unresponsive to classic treatments. Lymphoproliferative disorders may accompany to Schnitzler syndrome. Although there are many kinds of treatments, response to anakinra is well. Our case was 74 years old man presented with urticarial papule and plaques on his upper part of body and proximal part of extremities. Fever, arthralgia and neutropenia were together with his lesions. Patient was used systemic steroid and antihistaminic with diagnosis of urtica. Schnitzler syndrome was diagnosed. We decided to represent this case because Schnitzler syndrome accompanied to multiple myeloma is very rare in literature and diagnosis is often delayed. Key words: Multiple Myeloma; Schnitzler Syndrome.

Keywords

• Multiple Myeloma; Schnitzler Syndrome.

Multipl Miyeloma Eşlik Eden Schnitzler Sendromu: Bir Olgu Sunumu

Öz

hepatosplenomegali, lökositoz ve eritrosit sedimentasyon oran yüksekliği ile karakterize nadir görülen otoimmün bir hastalıktır. Uzun süren ve klasik tedavilere cevapsız kronik ürtikerde Schnitzler sendromu incelenmelidir. Schnitzler sendromuna lenfoproliferatif hastalıklar eşlik edebilir. Çeşitli tedavileri bulunmakla birlikte anakinraya yanıt iyidir. Olgumuz 5 yıldır gövde üst kısmı ve ekstremitelerin proksimallerinde ürtikeryal papül ve plakları olan 74 yaşında erkek hastaydı. Hastanın lezyonlarına ateş, artralji ve nötropeni eşlik ediyordu. Hasta ürtiker tanısı ile sistemik steroid ve antihistaminik kullanmıştır. Schnitzler sendromu tanısı konuldu. Literatürde multipl miyeloma eşlik eden Schnitzler sendromuna nadir rastlandığı ve sıklıklı tanı konulmasında gecikildiği için bu vakayı sunmayı uygun gördük. Anahtar kelimeler: Multipl Miyelom; Schnitzler Sendromu.

Anahtar Kelimeler

• Multipl Miyelom; Schnitzler Sendromu.

References

1. Lipsker D, Veran Y, Grunenberger F, Cribier B, Heid E, Grosshans E. The Schnitzler syndrome: Four new cases and review of the literature. Medicine 2001;80:37-44.
2. Lipsker D. The Schintzler Syndrome. Orphanet J Rare Dis. 2010;5:38.
3. De Koning HD, Bodar EJ, Van Der Meer J, 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 2007;37:137-48.
4. Sperr WR, Natter S, Baghestanian M, Smolen J, Wolff K, Binder BR, et al. Autoantibody reactivity in a case of Schnitler's syndrome: evidence for a Th1-like response and www.totmdergisi.org detection of IgG2 antiFcRIα antibodies. Int Arch Allergy Immunol 2000;122:279-86.
5. 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. J Allergy Clin Immunol 1991;88:244-56.
6. Eiling E, Schröder JO, Gross WL, Kreiselmajer I, Mrowietz U, Schwarz T. The Schnitzler' syndrome: chronic urticaria and monoclonal gammopathy an autoinflammatory syndrome? J Dtsch Dermatol Ges 2008;6:626-31.
7. Akimoto R, Yoshida M, Matsuda R, Miyasaka K, Itoh M. Schnitzler's syndrome with IgG kappa gammopathy. J Dermatol 2002;29:735-8.
8. De Waele S, Lecouvet FE, Malghem J, Jamar F, Lambert M. Schnitzler’s syndrome: an unusual cause of bone pain with suggestive imaging features AJR 2000;175:1325-27.

9. Tinazzi E, Pucetti A, Patuzzo G, Sorleto M, Barbieri A, Lunardi C, Schnitzler syndrome, and autoimmune- autoinflammatory syndrome: report of two new cases and review of the literature. Autoimmunity reviews 2011;10:404- 9.
10. 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;1289-95.