A Rare but Fascinating Disorder: Case Collection of Patients with Schnitzler Syndrome.

BACKGROUND: Schnitzler syndrome is a rare disorder characterized by a chronic urticarial rash and monoclonal gammopathy (IgM in more than 90% of the cases). It is difficult to distinguish from other neutrophilic urticarial dermatoses, and diagnosis is based on the Strasbourg criteria. Interleukin-1 is considered the key mediator, and interleukin-1 inhibitors are considered first line treatment. Here, we present two cases of Schnitzler syndrome, both successfully treated with anakinra. OBJECTIVES: To increase awareness regarding clinical presentation, diagnosis, and treatment of this rare disorder. CASES: We describe the clinical features and disease course of two patients with Schnitzler syndrome, diagnosed using the Strasbourg criteria. Both were treated with anakinra with remarkable response to therapy. CONCLUSION: Schnitzler syndrome is a rare and underdiagnosed disorder. High suspicion should be maintained in patients with chronic urticaria-like dermatoses, intermittent fevers, and arthralgias. A serum protein electrophoresis and immunofixation should be performed in these patients. The diagnosis is important to recognize as Schnitzler syndrome is associated with malignancy. A lymphoproliferative disorder develops in about 20% of patients at an average of 7.6 years after onset of symptoms. Thus, patients warrant long-term follow-up. IL-1 inhibitors are extremely effective in relieving symptoms and are considered first line therapy.

Development of Eosinophilic Fasciitis during Infliximab Therapy for Psoriatic Arthritis.

Eosinophilic fasciitis (EF) is a rare disorder involving chronic inflammation of the fascia and connective tissue surrounding muscles, nerves, and blood vessels. While its pathogenesis is not entirely understood, this disorder is thought to be autoimmune or allergic in nature. We present here a case of a 59-year-old male who developed peripheral eosinophilia and subsequent eosinophilic fasciitis during treatment with infliximab. To our knowledge, eosinophilic fasciitis has not been previously described in patients during treatment with an inhibitor of tumor necrosis factor α.

Ocular toxicity of hydroxychloroquine.

This review summarizes the current literature regarding the ocular complications of hydroxychloroquine. Hydroxychloroquine has been used since the 1950s for the treatment of various rheumatic and dermatologic diseases. Hydroxychloroquine can cause ocular toxicity, with the most serious being an irreversible retinopathy. At the present time, no "gold standard" exists for identification of the ocular toxicity prior to its development. This has led to controversy regarding the recommendations for ophthalmologic examinations for screening patients on hydroxychloroquine.

Giant cell arteritis.

This review summarizes the diagnosis, clinical manifestations and management of giant cell arteritis. Giant cell arteritis is an immune-mediated vasculitis of medium to large sized arteries that affects individuals older than the age of 50. Patients typically present with signs of vascular insufficiency of the extracranial arteries of the head and systemic inflammation. Temporal artery biopsy remains the hallmark of diagnosis. Specific treatment regimens must be tailored to each individual, but steroids remain the backbone of therapy.