Syndrome de Schnitzler: une cause rare d'urticaire systémique

Germain, Philippe; Fach, J; Nb, Bui; Traissac, T.; Delbrel, X.; Bras, M. Le; Longy‐Boursier, M.

doi:10.1016/s0248-8663(00)80049-1

Cited by 18 publications

(4 citation statements)

References 8 publications

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“…Osteocondensation is the most frequent radiological finding and the most common radiological pattern is a sclerotic bone marrow involvement with cortical hyperostosis of distal femora and proximal tibiae, and a lack of suggestive signs of malignancy. Lytic lesions were reported [26,27] as well as periosteal apposition [24,28]. Radiological differential diagnosis is broad and includes mastocytosis, POEMS syndrome, Erdheim-Chester disease, Camurati-Englemann or Van Buchem disease, Buschke-Ollendorf syndrome, osteopetrosis, melorheostosis, ribbing disease and hypertrophic osteoarthropathy.…”

Section: Introductionmentioning

confidence: 99%

“…Bone technetium scanning reveals hyperfixation in the areas of radiological involvement [24]. Magnetic resonance imaging confirms thickening of cortices and can show medullar bone involvement and marrow infiltration without space occupying features in the affected areas [24,28,29]. A solitary sclerotic lesion has been reported [30] and thus radiological differential diagnosis should be expanded to include entities as lymphoma, multiple myeloma, SAPHO syndrome, osteoblastic metastases, osteosarcoma, chronic osteomyelitis, osteoid osteoma, and healing stress fractures [31].…”

Section: Introductionmentioning

confidence: 99%

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The Schnitzler syndrome

Lipsker

2010

Orphanet J Rare Dis

154

168

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The Schnitzler syndrome is a rare and underdiagnosed entity which is considered today as being a paradigm of an acquired/late onset auto-inflammatory disease. It associates a chronic urticarial skin rash, corresponding from the clinico-pathological viewpoint to a neutrophilic urticarial dermatosis, a monoclonal IgM component and at least 2 of the following signs: fever, joint and/or bone pain, enlarged lymph nodes, spleen and/or liver, increased ESR, increased neutrophil count, abnormal bone imaging findings. It is a chronic disease with only one known case of spontaneous remission. Except of the severe alteration of quality of life related mainly to the rash, fever and pain, complications include severe inflammatory anemia and AA amyloidosis. About 20% of patients will develop a lymphoproliferative disorder, mainly Waldenström disease and lymphoma, a percentage close to other patients with IgM MGUS. It was exceedingly difficult to treat patients with this syndrome until the IL-1 receptor antagonist anakinra became available. Anakinra allows a complete control of all signs within hours after the first injection, but patients need continuous treatment with daily injections.In many aspects, the Schnitzler syndrome resembles the genetically determined auto-inflammatory syndromes involving activating mutations of the NLRP3 inflammasome. This latter point and its consequences will be addressed.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

The Schnitzler syndrome

Lipsker

2010

Orphanet J Rare Dis

154

168

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“…9 If MRI is performed, this typically shows evidence of cortical thickening as well as medullary bone involvement in the absence of a tumor. 15,16 Sometimes, the abnormality is restricted to the medulla with T 2 hyperintensity.…”

Section: Imaging Abnormalitiesmentioning

confidence: 99%

Schnitzler syndrome: clinical features and histopathology

Dingli¹,

Camilleri²

2015

PLMI

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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.

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“…Colchicine at the dose of 1 --2 mg/day can be truly effective in some patients (~20% of them), with the advantage of a favorable tolerance profile [13,[18][19][20]. Dapsone is usually ineffective [4].…”

Section: Inhibitors Of Neutrophilsmentioning

confidence: 99%