A 49-year-old Caucasian female presented with generalized arthralgias and recurrent diffuse pruritic maculopapular lesions over her face, torso, and upper extremities. The patient suffered outbreaks with variable intensity almost in a daily basis over the past five years with no identifiable triggering factors.
The patient mentioned that the lesions cleared up in 24 hours, leaving no marks or scars, while new lesions appeared daily.
Her past medical history was significant for transfusion related hepatitis C, diabetes mellitus type 2, depression, chronic pancreatitis, and malnutrition. 
Previous surgeries included pancreatic cyst removal at age 11, tonsillectomy/adenoidectomy, tubal ligation, and appendectomy.
She had a 35 pack/year history of smoking, with remote history of inhaled crack cocaine use. She denied alcohol or other illicit drug use.
Review of systems revealed unintended weight loss of about 20 pounds over a 6-month period, diffuse abdominal pain, generalized weakness, night sweats, and subjective recurrent low-grade fevers not associated with the onset of her skin lesions.
Her family history was noncontributory (father deceased at the age of 70 because of heart disease, mother had diabetes mellitus and coronary artery disease, and her brother and daughter had good health).
The initial physical examination revealed diffuse pruritic maculopapular lesions over her face, thorax, and upper extremities (Figure 1), sparing her palms, soles, and mucous membranes. Also mild hepatosplenomegaly was found.
Her laboratory studies showed marked leukocytosis of 48.6 with neutrophils of 97.8% with elevated D-dimer and erythrocyte sedimentation rate (ESR) (Table 1).
She was started on broad-spectrum antibiotics that were discontinued 72 hours after the admission when infections were ruled out. 
Antinuclear antibodies, rheumatoid factor, anticitrullinated peptide antibodies, human immunodeficiency virus enzyme-linked immunosorbent assay (ELISA), Venereal Disease Research Laboratory (VDRL), hepatitis B surface antigen (HBsAg), hepatitis B core antigen (HBcAg), hepatitis B e antigen (HBeAg), and cryoglobulins were found to be negative or within normal limits.
Serum protein electrophoresis showed monoclonal gammopathy; SPEP showed gamma region asymmetric spike consistent with monoclonal gammopathy. UPEP showed oligoclonal gammopathy involving IgG, IgM kappa, and lambda (IgM: 552 mg/L).
Biopsy of her skin lesions showed neutrophil rich cells admixed with eosinophils consistent with chronic urticaria. A right groin excisional lymph node biopsy showed reactive lymphoid hyperplasia with plasmacytosis.
Bone marrow biopsy showed a hypercellular marrow with plasmacytosis and myeloid predominance and was reported as negative for malignancy. 
Based on the clinical presentation and laboratory results the diagnosis of Schnitzler syndrome was made. As proposed by Lipsker et al. the diagnosis of this entity requires 2 major criteria and a minimum of 2 minor criteria. She had 2 major criteria (chronic urticarial rash/intermittent fever and monoclonal IgM) plus at least 5 minor criteria (arthralgias, bone pain, lymphoadenopathy, hepato- and/or splenomegaly, and elevated ESR and/or leukocytosis).
She was started on high dose of corticosteroids, nonsteroidal anti-inflammatory drugs, and antihistamines with partial remission of her symptoms and she was discharged home (Figure 2).
Anakinra was added to her regimen for several weeks with an excellent response; however due to economical constrains treatment was discontinued, and her symptoms partially recurred.