Essential Thrombocythemia's Role in the Complex Landscape of Vasculitis: A Case Report

Peripheral gangrenes have been sporadically documented as the initial presentation of essential thrombocythemia (ET), as exemplified in the preceding case reports. Nevertheless, the prevalence of vasculitis-induced skin problems as the primary indication of ET has not been extensively examined. Our case distinguishes itself in this regard, as it proposes a fresh outlook on the etiology of ET. A 52-year-old male presented with worsening pain and bruising in the right toes, following auto-amputation of the left fourth fingertip due to gangrene a month earlier. Examination revealed necrotic wounds on multiple toes. Dorsalis pedis pulses were palpable, and sonography ruled out obstructions. Splenomegaly and thrombocytosis prompted consideration of ET. Marrow analysis confirmed myeloproliferative neoplasia with negative JAK2, CALR, and MPL mutations. Axonal polyneuropathy was evident, and skin biopsy showed leukocytoclastic vasculitis (LCV). The patient was diagnosed with undifferentiated vasculitis, characterized by total signs of ischemia and gangrene of the fingertips, splenomegaly, triple-negative ET, neuropathy (medium vessel involvement), and cutaneous LCV (small vessel involvement). The patient received prednisolone at a dosage of 1 mg/kg and azathioprine concurrently with hydroxyurea, aspirin, cilostazol, and sildenafil. The results indicate a fresh perspective, suggesting that ET may potentially contribute to the development of LCV. The main takeaway lesson from this atypical manifestation highlights the wide range of clinical manifestations associated with ET and encourages further investigation into the complex relationship between thrombocythemia and vasculitis processes. As we delve deeper into these distinct presentations, we gain a more comprehensive understanding of the diverse aspects of ET pathology.