School of Medicine and Health Sciences Poster Presentations

Title

Purpuric Lesions and Non-Healing Ulcerations: An Unusual Presentation of Multiple Myeloma

Poster Number

175

Document Type

Poster

Status

Medical Student

Abstract Category

Clinical Specialties

Keywords

cryoglobulinemic vasculitis, multiple myeloma

Publication Date

Spring 2018

Abstract

Cryoglobulinemic vasculitis is a small vessel vasculitis that can be associated with various pathologies, including infection, autoimmunity, and malignancy. Diagnosing cryoglobulinemic vasculitis can be challenging due to its widely varying presentation and limited sensitivity with isolated laboratory testing. Here we present a case of cryoglobulinemic vasculitis associated with multiple myeloma.

The patient is a 64 year old woman with a past medical history of type 2 diabetes, hypertension, hyperlipidemia, hypothyroidism, asthma, and atrial fibrillation. She had a prior history of lower extremity purpuric lesions and ulcerations that responded to high dose steroids many years back. Several skin biopsies revealed both cutaneous vasculitis and thrombotic vasculopathy, an initial bone marrow biopsy was non-diagnostic, and labs indicated a worsening paraproteinemia on UPEP and positive serum cryoglobulins. She presented to an outside hospital with altered mentation, acute renal failure, and suspected pneumonia and cellulitis, and was later transferred to our hospital for further evaluation of progressive necrotic wounds.

On presentation, the patient’s physical exam revealed necrotic tissue of the bilateral knees, fixed areas of non-blanching reticular reddish-blue to purple discoloration of both upper and lower extremities, and scattered petechiae, palpable purpura, and lower extremity ulcers. Labs were significant for the presence of serum cryoglobulins, elevated serum free kappa to lambda light chain ratio, serum IFE with hypogammaglobulinemia, urine IFE with prominent M spikes and IgG monoclonal protein with kappa light chain specificity, Hgb of 6.5, platelets of 146, peak creatinine of 1.8, prolonged PT of 16.5 and INR of 1.34, and a UA with proteinuria.

Rheumatology and Hematology-Oncology were consulted during this admission, and a bone marrow biopsy revealed kappa light chain multiple myeloma as the underlying etiology of the patient’s Type 1 cryoglobulinemic vasculitis. A regimen of cyclophosphamide, bortezomib, and dexamethasone was initiated, however treatment was complicated by septic shock and encephalopathy, and ultimately the patient died from complications of her disease.

This case demonstrates the importance of maintaining a broad differential in the setting of worsening cutaneous vasculitis and multi organ failure that includes cryoglobulinemia. When serum cryoglobulins are detected, a thorough evaluation for underlying causes including infectious, rheumatologic, and hematologic etiologies should be pursued. Often, initial evaluation may not yield a cause of cryoglobulinemia. Given that long-term prognosis relies upon rapid diagnosis and treatment of the underlying disease process, close interval follow up and consideration of repeat investigation when paraproteinemia progresses is crucial.

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Purpuric Lesions and Non-Healing Ulcerations: An Unusual Presentation of Multiple Myeloma

Cryoglobulinemic vasculitis is a small vessel vasculitis that can be associated with various pathologies, including infection, autoimmunity, and malignancy. Diagnosing cryoglobulinemic vasculitis can be challenging due to its widely varying presentation and limited sensitivity with isolated laboratory testing. Here we present a case of cryoglobulinemic vasculitis associated with multiple myeloma.

The patient is a 64 year old woman with a past medical history of type 2 diabetes, hypertension, hyperlipidemia, hypothyroidism, asthma, and atrial fibrillation. She had a prior history of lower extremity purpuric lesions and ulcerations that responded to high dose steroids many years back. Several skin biopsies revealed both cutaneous vasculitis and thrombotic vasculopathy, an initial bone marrow biopsy was non-diagnostic, and labs indicated a worsening paraproteinemia on UPEP and positive serum cryoglobulins. She presented to an outside hospital with altered mentation, acute renal failure, and suspected pneumonia and cellulitis, and was later transferred to our hospital for further evaluation of progressive necrotic wounds.

On presentation, the patient’s physical exam revealed necrotic tissue of the bilateral knees, fixed areas of non-blanching reticular reddish-blue to purple discoloration of both upper and lower extremities, and scattered petechiae, palpable purpura, and lower extremity ulcers. Labs were significant for the presence of serum cryoglobulins, elevated serum free kappa to lambda light chain ratio, serum IFE with hypogammaglobulinemia, urine IFE with prominent M spikes and IgG monoclonal protein with kappa light chain specificity, Hgb of 6.5, platelets of 146, peak creatinine of 1.8, prolonged PT of 16.5 and INR of 1.34, and a UA with proteinuria.

Rheumatology and Hematology-Oncology were consulted during this admission, and a bone marrow biopsy revealed kappa light chain multiple myeloma as the underlying etiology of the patient’s Type 1 cryoglobulinemic vasculitis. A regimen of cyclophosphamide, bortezomib, and dexamethasone was initiated, however treatment was complicated by septic shock and encephalopathy, and ultimately the patient died from complications of her disease.

This case demonstrates the importance of maintaining a broad differential in the setting of worsening cutaneous vasculitis and multi organ failure that includes cryoglobulinemia. When serum cryoglobulins are detected, a thorough evaluation for underlying causes including infectious, rheumatologic, and hematologic etiologies should be pursued. Often, initial evaluation may not yield a cause of cryoglobulinemia. Given that long-term prognosis relies upon rapid diagnosis and treatment of the underlying disease process, close interval follow up and consideration of repeat investigation when paraproteinemia progresses is crucial.