- Schnitzler syndrome
Infobox Disease
Name = PAGENAME
Caption =
DiseasesDB = 31345
ICD10 =
ICD9 =
ICDO =
OMIM =
MedlinePlus =
eMedicineSubj = derm
eMedicineTopic = 489
MeshID = D019873Schnitzler syndrome is a
rare disease characterised bychronic hives (urticaria) and periodicfever ,bone pain and joint pain (sometimes with joint inflammation),weight loss ,malaise ,fatigue ,swollen lymph glands and enlarged spleen and liver.cite journal |author=de Koning HD, Bodar EJ, van der Meer JW, Simon A |title=Schnitzler syndrome: beyond the case reports: review and follow-up of 94 patients with an emphasis on prognosis and treatment |journal=Seminars in arthritis and rheumatism |volume=37 |issue=3 |pages=137–48 |year=2007 |month=December |pmid=17586002 |doi=10.1016/j.semarthrit.2007.04.001 |url=http://linkinghub.elsevier.com/retrieve/pii/S0049-0172(07)00073-X]The urticarial rash is non-
itching in more than half of cases, which is unusual for hives. It is most prominent on the trunk, arms and legs, sparing the palms, soles, head and neck. Associatedangioedema has been reported in a few patients. A review of 94 cases found a mean age at onset of 51 years, and only four patients developed symptoms before the age of 35. The cause and disease mechanism of Schnitzler syndrome remain largely unknown.Schnitzler syndrome is considered an
autoimmune disorder . Chronic hives and a monoclonal gammopathy have been proposed as the major criteria, while the others represent minor criteria.cite journal |author=Lipsker D, Veran Y, Grunenberger F, Cribier B, Heid E, Grosshans E |title=The Schnitzler syndrome. Four new cases and review of the literature |journal=Medicine |volume=80 |issue=1 |pages=37–44 |year=2001 |month=January |pmid=11204501 |doi= |url=http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0025-7974&volume=80&issue=1&spage=37]Diagnosis
Blood test s show a high concentration of specificgamma-globulins (monoclonal gammopathy ) of theIgM type. It almost always has light chains of the κ-type. A variant in whichIgG is raised has been described, which appears to be ten times as rare. The immunoglobulins may show up in the urine asBence Jones protein s. Signs of inflammation are often present: these include an increasedwhite blood cell count (leukocytosis ) and a raisederythrocyte sedimentation rate andC-reactive protein . There can beanemia of chronic disease . Bone abnormalities can be seen on radiological imaging (often increased density orosteosclerosis ) orbiopsy .Because it is such a rare condition (less than 100 cases reported in the literature worldwide before 2008), it is important to rule out other conditions which can cause periodic fevers, paraproteins or chronic hives. These include (and are not limited to) auto-immune or -inflammatory disorders such as adult-onset
Still's disease ,angioedema , hematological disorders such aslymphoma ormonoclonal gammopathy of undetermined significance , other causes of hives,cryoglobulinemia ,mastocytosis , chronicneonatal onset multisystem inflammatory disease orMuckle-Wells syndrome .Prognosis
The life span in patients with Schnitzler syndrome has not been shown to differ much from the general population. Careful follow-up is advised, however. A significant proportion of patients develops a lymphoproliferative disorder as a complication, most commonly
Waldenström's macroglobulinemia . This may lead to symptoms ofhyperviscosity syndrome .AA amyloidosis has also been reported in people with Schnitzler syndrome.Treatment
Antihistamines are not effective in treating the hives in this condition. It may respond toimmunosuppressant drugs such ascorticosteroids ,cyclooxygenase inhibitors ,interferon alpha ,interleukin 1 receptor antagonist s (Anakinra ),cite journal |author=de Koning HD, Bodar EJ, Simon A, van der Hilst JC, Netea MG, van der Meer JW |title=Beneficial response to anakinra and thalidomide in Schnitzler's syndrome |journal=Annals of the rheumatic diseases |volume=65 |issue=4 |pages=542–4 |year=2006 |month=April |pmid=16096327 |doi=10.1136/ard.2005.045245 |url=http://ard.bmj.com/cgi/pmidlookup?view=long&pmid=16096327]colchicine ,cyclosporine orthalidomide . The hives may respond to treatment withPUVA , and the bone pain may respond tobisphosphonates .History
The disease is named after the French dermatologist L. Schnitzler who first described this
syndrome in 1972.L. Schnitzler, Lésions urticariennes chroniques permanentes (érythème pétaloïde?) Cas cliniques No 46 B, J Dermatol Angers (1972) Abstract 46.]Footnotes
Wikimedia Foundation. 2010.