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Background: Schnitzler syndrome is characterized by chronic urticarial rash and monoclonal IgM gammopathy and is sometimes associated with periodic fever, arthralgias, and bone pain. Current treatment is unsatisfactory., Observations: Eleven patients with Schnitzler syndrome were treated with oral pefloxacin mesylate (800 mg/d). In 10 patients, we observed a dramatic and sustained improvement of urticarial and systemic manifestations. Corticosteroid therapy could be stopped or reduced in 6 patients. In 9 patients, pefloxacin was administered for more than 6 months (

Objective: To evaluate the prevalence of IgA and IgG antineutrophil cytoplasmic antibodies (ANCAs) in erythema elevatum diutinum in comparison with 2 other groups of neutrophilic dermatoses: Sweet syndrome and pyoderma gangrenosum., Design: Detection of IgA and IgG ANCAs in the serum of patients with neutrophilic dermatoses and characterization of the previously known antigenic targets., Setting: All serum was analyzed without knowledge of diagnosis in the Immunology Department, Pitié-Salpêtrière Hospital, Paris, France., Patients: Ten patients with erythema elevatum diutinum, 10 with Sweet syndrome, 10 with pyoderma gangrenosum, and 10 healthy volunteers., Main Outcome Measures: IgA and IgG ANCAs were sought by indirect immunofluorescence with ethanol and formaldehyde-fixed human neutrophil preparations as the substrate. Enzyme-linked immunosorbent assays were further performed for antigen characterization., Results: IgA ANCAs were observed in 60% and IgG ANCAs in 10 (33%) of the patients. All patients with erythema elevatum diutinum had IgA ANCAs. IgA fluorescence in formaldehyde-fixed neutrophils was restricted to those from patients with erythema elevatum diutinum. Enzyme-linked immunosorbent assays disclosed no single predominant target, and antigens remained largely undetermined in erythema elevatum diutinum., Conclusions: The ANCAs, particularly of IgA class, may prove to be a helpful paraclinical marker in erythema elevatum diutinum and an interesting perspective for understanding the pathophysiology of the disease. The nature of the unidentified targets and the pathogenicity of ANCAs, however, remain to be assessed.

Background: The association between primary hyperparathyroidism (PHPT) and monoclonal gammopathy has been reported, but whether it is fortuitous remains unsettled. We conducted a prospective study to determine the prevalence of monoclonal gammopathies in patients with surgically proved PHPT., Methods: In 101 consecutive patients with PHPT, serum immunoglobulins were systematically studied using agarose gel electrophoresis and immunofixation before and, when appropriate, after parathyroid surgery. The PHPT population was compared with a control series of patients with other diseases requiring surgery and with a group of patients with benign disease of the thyroid gland matched for age and sex to the PHPT population., Results: Monoclonal immunoglobulin was detected in 10 (10%) of 101 patients with PHPT (including 2 with multiple myeloma) compared with 2 (2%) of 127 patients who underwent other surgery (P =.005) and 3 (3%) of 101 patients with benign thyroid diseases (P =.04)., Conclusions: The prevalence of monoclonal gammopathies is high in patients with PHPT. At minimum, sensitive serum protein electrophoresis should be performed routinely in all patients with PHPT. Conversely, in patients with monoclonal gammopathy who have hypercalcemia but no other symptoms of progressive disease, clinicians must seek PHPT.