Clinical manifestation

Primary (essential) form: unassociated with underlying disease; secondary form: associated most commonly with internal malignancies and thromboembolic disease, but also with rheumatic diseases, diabetes mellitus, and pregnancy; purpura; ecchymoses; cutaneous gangrene; persistent, painful ulcerations, with surrounding livedo reticularis

Differential diagnosis Cryoglobulinemia; benign pigmented purpura; antiphospholipid antibody syndrome; Churg-Strauss syndrome; polyarteritis nodosa; serum sickness; Waldenstrom hyperglobulinemia; septic vasculitis; systemic lupus erythematosus; sarcoidosis

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