Sunil Narayan Tolat; Anil Gosavi; Vasudha Belgaumkar; Ravindranath Chavan; Nitika Deshmukh; Sweta Tharewal; Nupur Warke
Abstract
Schnitzler’s syndrome is an autoinflammatory disorder presenting with wheals, monoclonal gammopathy, and signs of inflammation. A 55-year-old woman presented with reddish, moderately itchy wheals with intermittent fever and arthralgia for two years. Multiple erythematous, edematous plaques were ...
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Schnitzler’s syndrome is an autoinflammatory disorder presenting with wheals, monoclonal gammopathy, and signs of inflammation. A 55-year-old woman presented with reddish, moderately itchy wheals with intermittent fever and arthralgia for two years. Multiple erythematous, edematous plaques were noted all over the body. Dermographism was present. A diagnosis of chronic urticaria was considered and treated with antihistamines. The patient returned six weeks later with partial symptomatic relief. She was then investigated for the cause of chronic urticaria, and the following differentials were considered: systemic lupus erythematosus (SLE), dermatomyositis, urticarial vasculitis, and auto-inflammatory diseases. The patient was febrile, and her blood investigations revealed leukocytosis and a raised erythrocyte sedimentation rate along with IgM gammaglobulinemia and an M band on serum electrophoresis. Skin biopsy revealed a neutrophilic infiltrate in the dermis. Thus, based on the Strasbourg diagnostic criteria, a final diagnosis of Schnitzler syndrome was made. Urticarial rash is one of the most common complaints encountered by dermatologists. Other extremely uncommon diseases like autoinflammatory disorders (for example, Schnitzler syndrome) can mimic chronic urticaria. The appearance of the rash and associated symptoms should be carefully considered to identify these missed cases. Auto-inflammatory syndromes are severely debilitating, with little awareness among healthcare professionals. Thus, they are often recognized with a diagnostic delay of many years. Early diagnosis of such rare diseases is imperative for effective treatment and to prevent devastating long-term complications.
