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Updated: May 29, 2026

Granulocyte-dependent Autoantibody-induced Skin Blistering

Granulocyte-dependent Autoantibody-induced Skin Blistering

Published on: October 12, 2012

Schnitzler syndrome with cold-induced urticaria.

Anil Kurian¹, Jason K Lee, Peter Vadas

¹Department of Medicine, McMaster University, Hamilton, Ontario, Canada.

Journal of Dermatological Case Reports

|September 3, 2011

Summary

This study reports the first case of Schnitzler syndrome presenting with cold-induced physical urticaria. This rare condition, characterized by monoclonal gammopathy and urticaria, highlights the need for broader diagnostic considerations.

Keywords:

Schnitzler syndrome arthralgia cold-induced fever hepatosplenomegaly lymphadenopathy monoclonal gammopathy urticaria

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Published on: December 15, 2011

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Last Updated: May 29, 2026

Granulocyte-dependent Autoantibody-induced Skin Blistering

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Published on: October 12, 2012

Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis

Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis

Published on: December 15, 2011

Area of Science:

Immunology
Dermatology
Hematology

Background:

Schnitzler syndrome is a rare autoinflammatory disorder.
It is characterized by monoclonal gammopathy, urticaria, inflammation, recurrent fever, bone pain, and arthralgia.
Approximately 100 cases have been reported globally.

Observation:

A 43-year-old woman presented with a 7-year history of chronic urticaria, angioedema with anaphylaxis, monoclonal gammopathy, and bone pain.
Her urticaria was triggered by cooler temperatures, confirmed by a positive ice cube test for cold-induced urticaria.
Despite aggressive therapies, the patient remained symptomatic.

Findings:

This case represents the first documented instance of Schnitzler syndrome with cold-induced physical urticaria.
The patient's presentation expands the known clinical spectrum of Schnitzler syndrome variants.
The condition proved refractory to conventional treatments.

Implications:

Schnitzler syndrome is under-recognized, especially atypical presentations.
Differential diagnosis should include hematologic disorders.
Novel therapies, such as IL-1 receptor inhibitors, may offer therapeutic promise for refractory cases.