Clinical and histopathologic review of Schnitzler syndrome: The Mayo Clinic experience (1972-2011)

Sokumbi, Olayemi; Drage, Lisa A.; Peters, Margot S.

doi:10.1016/j.jaad.2012.04.027

Cited by 70 publications

(86 citation statements)

References 32 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…2,3 Rash (red macules, papules or plaques without itching, with burning sensation) is present in almost all patients with Schnitzler's syndrome and can occur on any part of the body. 5 Monoclonal IgM component is the second most important criteria; it is associated with kappa light chains in 90% of patients. Differential diagnostic procedure should be carried out to exclude lymphoproliferative disease such as multiple myeloma and Waldenstrom's macroglobulinemia.…”

Section: Discussionmentioning

confidence: 99%

“…3,5 General constitutional symptoms and their effect on patient's quality of life determine the treatment strategy -anakinra for patients with more severe clinical and laboratory signs and colchicine for less prominent clinical and laboratory signs of the disease. Although the mechanism of the disease is still unknown, the efficacy of IL-1 blockade suggests that Schnizler's syndrome is an IL-1 driven disorder.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Different Therapeutic Paths (Colchicine vs. Anakinra) in Two Patients With Schnitzler’s Syndrome

et al. 2016

View full text Add to dashboard Cite

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Different Therapeutic Paths (Colchicine vs. Anakinra) in Two Patients With Schnitzler’s Syndrome

et al. 2016

View full text Add to dashboard Cite

“…Although in some patients IgM deposits have been found in the skin, whether these are pathologic or a consequence of the inflammatory process is unclear. 7,10,17 The presence of monoclonal IgM deposits is also not invariable, and in one series, this was only present in 3 of 13 patients tested. 10 The skin rash is reminiscent of several inherited periodic fever syndromes, especially the autoinflammatory cryopyrin-associated periodic syndrome (CAPS).…”

Section: Pathophysiologymentioning

confidence: 91%

“…7,10,17 The presence of monoclonal IgM deposits is also not invariable, and in one series, this was only present in 3 of 13 patients tested. 10 The skin rash is reminiscent of several inherited periodic fever syndromes, especially the autoinflammatory cryopyrin-associated periodic syndrome (CAPS). 18,19 In the latter, a mutation in the NLRP3 is often found.…”

Section: Pathophysiologymentioning

confidence: 91%

“…Palpable lymphadenopathy is found in approximately 45% of patients, while enlargement of the liver and spleen are less common and found in at most a third of patients. [3][4][5][6]10 The swollen lymph nodes are mainly found in the axillae and groin, with cervical adenopathy being less common. Nodes can vary in size and be up to several centimeters in diameter.…”

Section: Organomegalymentioning

confidence: 99%

See 1 more Smart Citation

Schnitzler syndrome: clinical features and histopathology

Dingli¹,

Camilleri²

2015

PLMI

View full text Add to dashboard Cite

Schnitzler syndrome is a rare and underrecognized syndrome characterized by chronic urticaria, a monoclonal protein, and a variety of other symptoms, including fever, bone pain, organomegaly, and evidence of an acute phase response. Biopsy of an involved area of the skin shows a neutrophilic infiltrate without evidence of vasculitis or hemorrhage. Although the etiology of the syndrome is unknown, current evidence suggests this is an autoinflammatory syndrome. Recognition of this syndrome is critical since it is highly responsive to anakinra.

show abstract

Chronic urticarial eruption associated with monoclonal gammopathy

et al. 2015

View full text Add to dashboard Cite

Schnitzler syndrome is a rare acquired autoinflammatory disease characterized by monoclonal immunoglobulin M gammopathy and urticaria. Clinical features include fever, urticarial rash, myalgia, arthralgia, hepatosplenomegaly, and enlarged lymph nodes. Schnitzler syndrome is often underdiagnosed and not usually suspected until more common autoimmune, neoplastic, and infectious etiologies are excluded. Anakinra, an interleukin-1 receptor antagonist, is the first-line treatment and has been shown to provide fast and sustained symptom relief [1][2][3]. IMAGES IN HEMATOLOGY AJH AJHA 50-year-old man with presumed Castleman disease/POEMS syndrome (a multisystem syndrome combining polyneuropathy, organomegaly, endocrinopathy, M component, and skin changes) presented for evaluation of persistent hip pain and a "burning" rash of 3 years' duration. He had previously been treated with systemic corticosteroids, lenalidomide, rituximab, and bortezomib, and with an autologous stem cell transplant, resulting in initial improvement but eventual recurrence of symptoms. His physical examination revealed small, slightly edematous, pink plaques involving the back (Image 1A), trunk, and arms (Image 1B). Laboratory tests revealed elevated inflammatory markers, with an erythrocyte sedimentation rate of 75 mm/hr (reference range, 0-22 mm/hr) and C-reactive protein of 65 mg/L (reference range, 0-8 mg/L), and small monoclonal immunoglobulin M j protein. Magnetic resonance imaging demonstrated patchy intramedullary osteosclerosis of the left iliac wing, with bone marrow edema in the left acetabulum and both knees. Whole-body fludeoxyglucose (FDG) positron emission tomography/computed tomography revealed mild increased 18

show abstract

Clinical and histopathologic review of Schnitzler syndrome: The Mayo Clinic experience (1972-2011)

Cited by 70 publications

References 32 publications

Different Therapeutic Paths (Colchicine vs. Anakinra) in Two Patients With Schnitzler’s Syndrome

Different Therapeutic Paths (Colchicine vs. Anakinra) in Two Patients With Schnitzler’s Syndrome

Schnitzler syndrome: clinical features and histopathology

Chronic urticarial eruption associated with monoclonal gammopathy

Contact Info

Product

Resources

About