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A Rare Variant of Schnitzler Syndrome

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A Rare Variant of Schnitzler Syndrome A Rare Variant of Schnitzler Syndrome: A Case Study Lacey Beth Elwyn, DO*, Shawn Michael Walls, DO**, Zachary Jason Fischer***, Cindy Hoffman, DO****, Damian DiCostanzo, MD***** *Dermatology resident, OGME-IIII Saint Barnabas Hospital Department of Dermatology, Bronx, NY **Internal medicine resident, OGME-II, University Hospitals Regional Hospitals Richmond Medical Center Department of Medicine, Richmond Heights, OH ***Undergraduate, BS, Quinnipiac University, Department of Health Science, Hamden, CT ****Dermatology Residency Program Director, Saint Barnabas Hospital Department of Dermatology, Bronx, NY *****Dermatopathologist, Dermpath Diagnostics, Port Chester, NY Abstract Case Report Discussion Conclusion Schnitzler Syndrome is a rare auto-inflammatory disease characterized by A 51 year old Caucasian woman presented with an asymptomatic, chronic Schnitzler syndrome is a rare, under diagnosed disorder characterized by We report an atypical case of Schnitzler syndrome consisting of a chronic a chronic urticarial neutrophilic dermatosis and an IgM monoclonal red eruption, originally on her abdomen with extension centrifugally to chronic urticarial dermatosis, monoclonal gammopathy, and systemic urticarial neutrophilic dermatosis, an IgG and IgA kappa light chain gammopathy. We report a rare case of the syndrome consisting of a chronic proximal extremities that has remained stable for greater than 12 years. inflammation. A retrospective study at the Mayo Clinic highlighted that biclonal gammopathy, and multiple systemic inflammatory symptoms. In urticarial lymphocytic dermatosis, an IgG and IgA kappa light chain Past medical history includes osteoarthritis, anxiety, microcytic anemia, this disease is highly under-diagnosed by identifying 46 undiagnosed cases recent years, treatment with IL-1 receptor antagonist leads to complete biclonal gammopathy, and multiple systemic symptoms including fatigue, monoclonal gammopathy of undetermined significance and positive lupus by cross-referencing from their dysproteinemia data base with medical remission of the dermatologic manifestations and musculoskeletal pain in 22 arthralgias, and bone pain. For a decade, this patient suffered from anticoagulant. Review of systems positive for fatigue, arthralgia, and bone records from all patients with chronic urticaria at the institution . Nineteen patients with Schnitzler syndrome10. The malignant potential and available musculoskeletal pain and a persistent cutaneous eruption refractory to pain. Medications included Effexor 150mg with no known drug allergies. percent of reported patient’s with Schnitzler syndrome developed success in treatment, prompted reporting of this unusual case in hopes to 9 multiple pharmacologic interventions. This condition carried with it a Examination of her trunk revealed diffuse urticarial plaques (Figure 3a) lymphoproliferative disorders which highlights the importance of expand the differential diagnosis to consider Schnitzler syndrome in any history of multiple different biopsy confirmed diagnoses but ultimately and extremities revealed pale rose macules with few raised papules and recognizing the diagnosis and subsequent follow-up in these patient whom presents with a chronic urticarial dermatosis and monoclonal was diagnosed as a rare Schnitzler Syndrome variant. Subsequently, this plaques (Figure 3b). Tenderness to palpation was appreciated over the tibia patients. Liliane Schnitzler was the first to recognize and report the gammopathy. This patient is finally achieving resolution of symptoms and patient is achieving resolution of symptoms on the IL-1 receptor and iliac bones. Axillary lymphadenopathy was also present. Laboratory particular combination of chronic urticaria and a monoclonal gammopathy overall improvement in quality of life on an IL-1 receptor antagonist. antagonist, Kineret. We report this unusual case of probable Schnitzler studies: positive ANA 1:160, homogenous pattern, and negative reflex in 1972.21Schnitzler syndrome is a diagnosis of exclusion based on Syndrome in hopes to bring attention to the disease, both clinically and screen; normal complement C3, C4, and CH50;elevated p-ANCA 1:40; established diagnostic criteria originally presented by Lipsker et al in 2001 dermatopathologically, revisit its proposed pathophysiology, and consider normal ESR; positive for lupus anticoagulant, low positive for cardiolipin and later accepted by Koning et al in 2007 (Table 1.1). Our patient suffered the possibility of rare variations of this often overlooked syndrome. antibody; slightly elevated IgG and IgM titers; normal beta-2 from chronic urticarial dermopathy, biclonal gammopathy, and systemic References microglobulin; elevated PTT; microcytic anemia; stable IgG and IgA symptoms including lymphadenopathy, anemia, arthralgia, and bone pain. Introduction kappa monoclonal proteins on serum immunofixation with borderline high By definition, this patient was diagnosed with Schnitzler syndrome and is 1. Asero R, Tedeschi A, Coppola R, et al. Activation of the tissue factor pathway of blood coagulation in patients with chronic kappa/lambda ratio; free kappa monoclonal light chains in urine believed to have an atypical biclonal variant of the classic presentation. urticarial. J Allergy Clin Immunol. 2007;119:705-10. 2. Angles-Cano El et al. Antiphospholipid antibodies and the coagulation cascade. Rheum Dis Clin North Am. 2001 Schnitzler syndrome is a chronic auto-inflammatory disease with no immunofixation; Quantitative IgG, IgM, and IgA levels within normal Although IgM monoclonal gammopathy is the biological hallmark of the Aug;27(3):573-86. 4,5,6,21 3. Famularo et al. Severe thrombophilia with antiphospholipid syndrome and hyperhomocysteinemia in a patient with reported spontaneous remissions and a potential to progress into a limits. Skeletal survey negative for osteolytic lesions. This patient was disease, variants have been reported in <10% of cases . A literature Schnitzler's syndrome. Clin Exp Rheumatol. 2003 May-Jun;21(3):366-8. lymphoproliferative malignancy. Diagnosis requires chronic neutrophilic given the diagnosis of an atypical variant of Schnitzler syndrome and was search completed by de Koning revealed IgM kappa subtype in 85% of 4. Patel, S. et al. Chronic urticaria with monoclonal IgG gammopathy: a clinical variant of Schnitzler syndrome? Ann Allergy started on an IL-1 receptor antagonist at a dose of 1.2mg/kg/ day. After 1 patients21. Variant cases of IgG subtype constituted 7% of the reported Asthma Immunol. 109 (2012) 147-149. urticarial dermatosis (Figure 1), IgM monoclonal gammopathy, and at least 5. Carlesimo M. Et al. Chronic vasculitis urticaria associated 21 2 systemic inflammatory symptoms (Table 1.1). Rare variants of Schnitzler month of treatment, patient reported significant improvement in her pain cases and a biclonal gammopathy was present in 7 cases . We present the to a monoclonal gammopathy of IgM and IgA type, a Schnitzler Syndrome Eur J Dermatol. 2000 Nov-Dec;20(6):838-9. and dermatologic eruption (Figure 8). Complications of treatment included first case of a biclonal gammopathy including IgG kappa monoclonal 6. Carlesimo M, et al. Chronic urticaria and IgG paraproteinaemia: unusual spectrum of Schnitzler Syndrome. Clin Ter. syndrome, such as IgG monoclonal and IgA biclonal proteins are reported 2011;162(3):85-87. 21 in the literature . The most common histopathological feature of injection site reaction, which reportedly occur in 80% of patients with protein in addition to an IgA kappa monoclonal protein. IL-1 plays the 7. Doegkas HMG. Reactions to aspirin and food additives in patients with chronic urticaria, including physical urticarias. Br J Schnitzler syndrome is neutrophilic urticaria with intact vasculature and average resolution over 1-2 months. Her injection reactions were major role in the pathophysiology of Schnitzler syndrome. The Dermatol. 1975;93:135-43. 8. Bizzaro, N. et al. False-positive results for IgA anti-phospholipid antibodies in patients with IgA monoclonal gammapthy. mild papillary dermal edema (Figure 2). The histopathological differential controlled with topical clocortolone cream and oral antihistamines. dermatologic manifestation is a mast cell independent urticarial Clinical Chemisstry 45, No. 11, 1999. 18 dermatosis. A local inflammatory response, via IL-1, is thought to induce 9. Kyle RA et al. Long term follow-up of IgM monoclonal gammopathy of undetermined significance. Blood 2003, diagnosis includes neutrophilic urticarial dermatoses (Table 2). The Dermatopathology 102(10):3759-64. patho-mechanism of Schnitzler syndrome is reported to involve the Multiple punch biopsies revealed sparse superficial perivascular the skin lesions. It is postulated that mutations of genes in the IL-1 10. Gilson M, et al. Treatment of Schnitzler syndrome with anakinra. Clin Exp Rheumatol 2007, 25:931. activation of inflammasome, IL-1. IL-1 receptor inhibition is the pathway may be responsible for disease21. Currently, the majority of data 11. Tohyama M, et al. A marked increase in serum soluble Fas ligand in drug-induced hypersensitivity syndrome. Br J lymphocytic infiltrate with mild papillary dermal edema, suggestive of Dermatol 2008; 159:981-4. best-known treatment for Schnitzler syndrome17. urticaria. (Figures 4, 5, 6). The most recent biopsy was taken from the left supports that the monoclonal gammopathy is caused by the systemic 12. Guicciardi ME, et al. Apoptosis: a mechanism of acute and chronic liver injury. Gut 2005; 54:1024-22. inflammation21.
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