SHM Converge Scientific Abstract ePoster Gallery-Rashes and Red Cells: A Tale of Two Conditions

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This case describes a 55-year-old woman with hypertension who presented with 5 days of fatigue and a rapidly spreading, pruritic, non-blanching purple-red rash on both legs. Examination showed tense purpuric lesions extending from the thighs downward, and initial labs revealed marked erythrocytosis and severe thrombocytosis. Peripheral smear confirmed increased red cells and platelets.<br /><br />Workup showed elevated IgA, low IgM, and otherwise largely normal inflammatory, autoimmune, and renal studies. Skin biopsy demonstrated leukocytoclastic vasculitis with IgA and C3 deposition, confirming IgA vasculitis. Because of the striking blood count abnormalities, a myeloproliferative neoplasm panel was sent and revealed a JAK2 V617F mutation, establishing a diagnosis of polycythemia vera (PV).<br /><br />The report emphasizes that coexisting IgA vasculitis and PV is rare. Published cases have mostly involved men and often included renal disease such as hematuria or proteinuria. Common associated features include hypertension, hepatosplenomegaly, flushing, conjunctival suffusion, visual changes, and palpable purpura.<br /><br />Key diagnostic points: IgA vasculitis is suggested by palpable purpura or petechiae without thrombocytopenia, plus features such as abdominal pain, renal involvement, or IgA-predominant leukocytoclastic vasculitis. PV is diagnosed by elevated hemoglobin/hematocrit with JAK2 mutation or bone marrow panmyelosis.<br /><br />Treatment differs for each condition. IgA vasculitis without renal involvement is usually supportive. PV is treated with phlebotomy to keep hematocrit below 45%, low-dose aspirin, and cytoreduction such as hydroxyurea; ruxolitinib may be used for hematologic control. In this patient, ruxolitinib improved both cytoses and skin lesions.<br /><br />The main takeaway is to consider dual pathology in patients with palpable purpura, hypertension, renal findings, or hepatosplenomegaly, since earlier recognition may prevent progression to renal disease.

Asset Subtitle

Evan Raff

Meta Tag

Author List Christian Cook, Donna Culton, Evan Raff, Jayson Miedema, Jiwoo Kim, Mukhtar Adem, Yuri Fedoriw

Category Clinical Vignettes

Concept IgA Vasculitis

Concept Polycythemia Vera

Concept Palpable Purpura

Concept JAK2 Testing

Concept Primary erythrocytosis

Distinguished Non-Finalist

Presenter Organization University of North Carolina School of Medicine

Presenting Author Evan Raff

Track Adult

Keywords

IgA vasculitis

polycythemia vera

JAK2 V617F

palpable purpura

leukocytoclastic vasculitis

thrombocytosis

erythrocytosis

C3 deposition

hydroxyurea

ruxolitinib

IgA Vasculitis

Polycythemia Vera

Palpable Purpura

JAK2 Testing

Primary erythrocytosis