Severe Retiform Purpura with Cryoglobulinemia

Introduction

Mixed cryoglobulinemia is a rare systemic vasculitis characterized by the presence of cryoglobulins precipitating at low temperatures. These proteins can impede blood flow, leading to a variety of symptoms such as purpura, skin ulcers, and multi-organ impairment in severe cases. This condition presents diagnostic challenges due to its variable clinical presentation and relatively rare nature. This report details the case of a 41-year-old African American female with end-stage renal disease (ESRD) on peritoneal dialysis, initially suspected to have calciphylaxis, who was ultimately diagnosed with mixed cryoglobulinemia.

Methods

The patient's clinical presentation of bilateral thigh and breast wounds associated with severe pain, prompted a thorough clinical workup. Punch biopsies were taken from areas of new retiform purpura and examined with H&E and Von Kossa staining. Extensive lab work and serological analysis were obtained to help identify the underlying etiology.

Results

Biopsy examination was indicative of vasculopathy and comprehensive serologic analysis detected quantitative cryoglobulins, confirming non-infectious mixed cryoglobulinemia. Despite therapeutic interventions, the patient succumbed to complications associated with ESRD and secondary infections.

Conclusion

This case highlights the complexity of problems seen in ESRD patients on dialysis. When presented with a patient with ESRD with retiform purpura in fatty areas, calciphylaxis should be considered. If refractory or severe, a search for additional causes should be performed. Given its relatively rare nature, physicians must have a high index of suspicion to consider mixed cryoglobulinemia. Secondary complications in ESRD patients with retiform purpura are common, and continued surveillance for infectious complications is recommended.