Schnitzler’s syndrome with prominent neutrophil infiltration misdiagnosed as Sweet’s syndrome: a typical example of urticarial neutrophilic dermatosis

Asahina, Akihiko; Sakurai, Natsuki; Suzuki, Yasuto; Narushima, Kazuo

doi:10.1111/j.1365-2230.2009.03746.x

Cited by 16 publications

(19 citation statements)

References 10 publications

(16 reference statements)

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“…Laboratory investigations reveal elevation of IL-1ß, IL-6, IL-18, ESR, and CRP levels. [154][155][156] Histology of lesional skin demonstrates a perivascular infiltrate consisting of lymphocytes, histiocytes, and neutrophils. 145 Immunofluorescence staining shows IgM deposits in the papillary dermis or at the basement membrane.…”

Section: Schnitzler Syndromementioning

confidence: 99%

Autoinflammation: From monogenic syndromes to common skin diseases

Nguyen

Cowen

Leslie

2013

Journal of the American Academy of Dermatology

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Autoinflammation is characterized by aberrant regulation of the innate immune system and often manifests as periodic fevers and systemic inflammation involving multiple organs, including the skin. Mutations leading to abnormal behavior or activity of the interleukin 1 beta (IL-1ß)-processing inflammasome complex have been found in several rare autoinflammatory syndromes, for which anticytokine therapy such as IL-1 or tumor necrosis factor-alfa inhibition may be effective. It is becoming clear that features of autoinflammation also affect common dermatoses, some of which were previously thought to be solely autoimmune in origin (eg, vitiligo, systemic lupus erythematosus). Recognizing the pathogenetic role of autoinflammation can open up new avenues for the targeted treatment of complex, inflammatory dermatoses.

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Section: Schnitzler Syndromementioning

confidence: 99%

Autoinflammation: From monogenic syndromes to common skin diseases

Nguyen

Cowen

Leslie

2013

Journal of the American Academy of Dermatology

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“…Schnitzler’s syndrome is a rare and acquired systemic disease which presents in mid-adulthood with non-pruritic urticaria-like exanthemas and IgM gammopathy, variably accompanied by intermittent fever, fatigue, arthritis or arthralgia, bone pain, lymphadenopathy and hepato- and/or splenomegaly, inflammatory anemia, persistent increase of neutrophils and thrombocytosis [38]. Associated findings include pseudoxanthum elasticum, pheripheral neuropathy, impairment of renal function, hearing loss and inflammatory amyloidosis.…”

Section: Multifactorial Autoinflammatory Diseases (Table 2)mentioning

confidence: 99%

An Update on Autoinflammatory Diseases

Ciccarelli¹,

Martinis²,

Ginaldi

2013

CMC

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Autoinflammatory diseases area group of clinical conditions other than autoimmune diseases, characterized by recurrent inflammatory episodes. From apathogenetic point of view they are determined by a dys regulation of innate immunity, without involvement of specific immunity (auto reactive T cells and auto antibodies). Recently, the increased knowledge in the field of auto inflammation highlighted shared immune mechanisms in the pathogenesis of both classical monogenetic and multifactorial auto inflammatory diseases and a broad spectrum of chronic age-related inflammatory pathologies. The current increase in the prevalence of chronic inflammatory diseases makes this subject of topical interest. In the light of these considerations, we propose an update of auto inflammatory diseases and a new interpretation of auto inflammation with both theoretical and clinical implications.

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“…Schnitzler syndrome is a rare disorder characterized by chronic urticarial rash and monoclonal gammopathy, along with fever, arthralgia, or bone pain [43]. Although the pathophysiology is presently unknown, the clinical improvement seen in these patients treated with IL-1 antagonists implies an autoinflammatory process [44].…”

Section: Differential Diagnosismentioning

confidence: 99%

Cryopyrin-Associated Periodic Syndrome: An Update on Diagnosis and Treatment Response

Leslie

2010

Curr Allergy Asthma Rep

113

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Cryopyrin-associated periodic syndrome (CAPS) is a rare hereditary inflammatory disorder encompassing a continuum of three phenotypes: familial cold autoinflammatory syndrome, Muckle-Wells syndrome, and neonatal-onset multisystem inflammatory disease. Distinguishing features include cutaneous, neurological, ophthalmologic, and rheumatologic manifestations. CAPS results from a gain-of-function mutation of the NLRP3 gene coding for cryopyrin, which forms intracellular protein complexes known as inflammasomes. Defects of the inflammasomes lead to overproduction of interleukin-1, resulting in inflammatory symptoms seen in CAPS. Diagnosis is often delayed and requires a thorough review of clinical symptoms. Remarkable advances in our understanding of the genetics and the molecular pathway that is responsible for the clinical phenotype of CAPS has led to the development of effective treatments. It also has become clear that the NLRP3 inflammasome plays a critical role in innate immune defense and therefore has wider implications for other inflammatory disease states.

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Schnitzler’s syndrome with prominent neutrophil infiltration misdiagnosed as Sweet’s syndrome: a typical example of urticarial neutrophilic dermatosis

Cited by 16 publications

References 10 publications

Autoinflammation: From monogenic syndromes to common skin diseases

Autoinflammation: From monogenic syndromes to common skin diseases

An Update on Autoinflammatory Diseases

Cryopyrin-Associated Periodic Syndrome: An Update on Diagnosis and Treatment Response

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