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More Than Skin Deep: A Systemic Cause of Leukocyto ...
More Than Skin Deep: A Systemic Cause of Leukocytoclastic Vasculitis
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This clinical vignette describes a 66-year-old man who presented with fevers, arthralgias, and fatigue in the setting of a recently diagnosed leukocytoclastic vasculitis (LCV) rash involving the skin. The rash was unusual because it was IgM/C3 positive and did not improve with steroids, raising concern for a systemic cause rather than typical self-limited LCV.<br /><br />Initial evaluation focused on infectious, autoimmune, and neurologic causes and was unrevealing: lumbar puncture studies were negative, broad infectious workup was negative, and autoimmune testing was negative. Inflammatory markers were elevated. Because of the patient’s persistent symptoms and steroid-refractory vasculitis, further workup was pursued.<br /><br />Subsequent testing revealed evidence of an underlying plasma cell disorder. Bone marrow biopsy showed 5–10% kappa-restricted plasma cells. Serum studies demonstrated an IgG kappa monoclonal protein with an M-spike of 0.4 g/dL, and cryoglobulin testing confirmed type I cryoglobulinemia. Imaging also found scattered lytic lesions, supporting the diagnosis of multiple myeloma.<br /><br />The final diagnosis was paraneoplastic type I cryoglobulinemia causing LCV secondary to IgG kappa multiple myeloma. The patient was treated with induction chemotherapy, followed by autologous hematopoietic stem cell transplantation. At six-month follow-up, the vasculitic rash had resolved and he had returned to baseline functional status.<br /><br />Key teaching points are that systemic symptoms in a patient with LCV—such as anemia, arthralgias, fatigue, or fever—should prompt evaluation for uncommon causes, including hematologic malignancy. Although LCV is usually benign and self-limited, rare cases can reflect type I cryoglobulinemia from a plasma cell dyscrasia. Treating the underlying malignancy can resolve the vasculitis.
Asset Subtitle
Austin M. Keller
Meta Tag
Author List
Austin M. Keller, Constanza Menendez Alurralde, Marion v. Stanley, Niraj v. Lawande
Category
Clinical Vignettes
Concept
Leukocytoclastic Vasculitis
Concept
Type I Cryoglobulinemia
Concept
Multiple Myeloma
Concept
Systemic Cause
Concept
Malignancy
Distinguished
Non-Finalist
Presenter Organization
Northwestern University Feinberg School of Medicine - Department of Medicine
Presenting Author
Austin M. Keller
Track
Adult
Keywords
leukocytoclastic vasculitis
type I cryoglobulinemia
multiple myeloma
IgG kappa monoclonal protein
plasma cell dyscrasia
paraneoplastic vasculitis
steroid-refractory rash
lytic bone lesions
bone marrow biopsy
autologous stem cell transplantation
Leukocytoclastic Vasculitis
Type I Cryoglobulinemia
Multiple Myeloma
Systemic Cause
Malignancy
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