Neurophilic urticarial dermatosis

Ehrchen, Jan

doi:10.1007/s00105-016-3788-0

Hautarzt

2016

DOI: 10.1007/s00105-016-3788-0

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Neurophilic urticarial dermatosis

Jan Ehrchen

Abstract: Neurophilic urticarial dermatosis (NUD) is a new, important differential diagnosis of urticarial rashes. It is a clinical and histological response pattern that strongly correlates with systemic disease. Both autoinflammatory and autoimmune conditions can be present in patients with NUD. In this article the clinical and histological criteria of NUD, diagnostic considerations and the diseases most frequently associated with NUD are discussed.

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Cited by 9 publications

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Schnitzler-Syndrom

Gellrich

Günther

2018

Hautarzt

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Schnitzler syndrome is a very rare acquired systemic disease with many similarities to hereditary autoinflammatory syndromes. The main characteristics are generalized exanthema and a monoclonal gammopathy with IgM. Other clinical features include fever, muscle, bone and/or joint pain, and lymphadenopathy. About 15-20% of patients with Schnitzler syndrome develop lymphoproliferative diseases and, in rare cases, amyloid A (AA) amyloidosis can occur if the disease is not treated. Activation of the innate immune system, especially interleukin(IL)-1β, is central in the pathogenesis of the disease. Consequently, complete control of disease symptoms can be achieved in 80% of patients by treatment with the IL-1 receptor antagonist anakinra.

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Schnitzler-Syndrom

Gellrich

Günther

2018

Hautarzt

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Schnitzler syndrome

Gellrich¹,

Günther²

2019

Hautarzt

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Autoinflammatorische Dermatosen

Ehrchen

Drerup

2020

Hautarzt

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Neurophilic urticarial dermatosis

Cited by 9 publications

References 17 publications

Schnitzler-Syndrom

Schnitzler-Syndrom

Schnitzler syndrome

Autoinflammatorische Dermatosen

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