A guide to recognise and manage Schnitzler syndrome in clinical practice

Schnitzler syndrome is a rare auto-inflammatory disease characterised by distinct skin manifestations. Although effective therapies are available, the pathogenesis of the condition remains only partially understood. Dr Heleen de Koning (Erasmus Medical Center, the Netherlands) discussed the diagnosis, clinical presentation, treatment options, and ongoing research into the underlying mechanisms of Schnitzler syndrome [1]. Diagnosis is based on 2 mandatory criteria: the presence of chronic urticarial dermatitis and a monoclonal IgM or IgG component. Additionally, patients frequently experience recurring fever, leucocytosis, increased CRP, objective signs of abnormal bone remodelling, bone pain, and a neutrophilic dermal infiltrate on skin biopsy. “For a definitive diagnosis, both mandatory criteria must be fulfilled, and at least 2 minor criteria in cases with IgM or at least 3 minor criteria in cases with IgG component,” said Dr de Koning. As of now,...

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Posted on 5 June 20255 June 2025