Essential thrombocythemia manifesting as livedoid and purpuric skin lesions: Report of two cases and literature review

Abstract

Essential thrombocythemia is a chronic myeloproliferative syndrome which usually runs its course as an asymptomatic elevated platelet count. Cutaneous manifestations secondary to microcirculation abnormalities are rare but can represent a helpful diagnostic clue in order to prevent major thromboembolic events. We report two cases of heterogeneous livedoid and "net-like" skin lesions in the context of essential thrombocythemia with identical histopathologic findings (medium-sized blood vessels with luminal obliteration by eosinophilic material, mostly positive for the platelet marker CD61, without vasculitis). In conclusion, we seek to raise awareness of the clinicopathological features of essential thrombocythemia to allow for prompt diagnosis and treatment.

Clinical, Histopathological and Molecular Spectrum of Cutaneous Lesions in Myelodysplastic Syndrome and Myeloproliferative Neoplasms (MDS/MPN): An Integrative Review

“…Other chronic myeloproliferative disorders associated with livedoid and purpuric cutaneous lesions due to microcirculation abnormalities are essential thrombocythemia [84,85]. Due to the elevated platelet count, the main histopathologic finding in these lesions consists of luminal obliteration by homogeneous eosinophilic material of medium-sized blood vessels.…”

“…Due to the elevated platelet count, the main histopathologic finding in these lesions consists of luminal obliteration by homogeneous eosinophilic material of medium-sized blood vessels. Immunohistochemistry demonstrates positivity for the platelet marker, CD61, in the eosinophilic material, without features of vasculitis, characteristic of occlusive vasculopathy [84,85].…”