Type I Cryoglobulinemic Vasculitis
A 67-year-old man with chronic lymphocytic leukemia (CLL) presented to the hematology clinic with a painful rash. Three years earlier, he had received a diagnosis of CLL with an associated IgG lambda monoclonal protein and had been closely monitored. Four months before the current presentation, the patient began having painful discoloration of his skin when he was exposed to temperatures of 3°C or less. He reported no fatigue, arthralgias, or neurologic symptoms. On physical examination, there was purple discoloration of the ear along with livedoid skin changes on the cheek (Panel A) and purpura on both calves (Panel B). A skin biopsy of the right leg showed leukocytoclastic vasculitis. Laboratory testing was notable for a low complement 4 level, normal complement 3 level, and a cryocrit level of 4% IgG lambda. Urinalysis results were normal. A diagnosis of type I cryoglobulinemic vasculitis secondary to CLL was made. Cryoglobulins — immunoglobulins that precipitate in cooled serum — can induce a small-vessel vasculitis that commonly manifests with cutaneous findings. The vasculitis is categorized as type I when it is associated with a monoclonal protein linked to a B-cell lymphoproliferative disorder. After 6 weeks of treatment with systemic chemotherapy and rituximab, the patient’s symptoms abated.