Vasculitic Leg Ulcer Revealing an Essential Thrombocythemia

A 64-year-old man was referred to our department due to painful ulcers on the right leg that had evolved over the previous 6 months. There was also progressive weight loss. He had no relevant medical history. Clinically, we observed multiple ulcers, some of them with a necrotic base, located over the medial malleolus and calcaneus (Figures 1 and 2), with an associated livedoid appearance of the dorsum of the feet. A skin biopsy revealed epidermis with ulceration. The superficial and deep dermis showed perivascular and interstitial neutrophilic inflammatory infiltrate, with fibrinoid necrosis of the vessels as well as leukocytoclasia (Figure 3). Blood analysis showed significant thrombocytosis (1128×10⁶ per µL) and leukocytosis (21.38×10⁶ per µL). The autoimmune study showed no abnormalities. Abdominal ultrasound showed hepatosplenomegaly. The patient was seen in the hematology department, and a bone marrow biopsy was obtained that was compatible with essential thrombocythemia (ET). The patient had a karyotype that showed no metaphases, and was BCR-ABL-negative and JAK2-positive. He started treatment with α-interferon 1.8 million units, three times per week. Daily polyacrylate wound dressing was carried out to debride the skin lesions, and there was gradual improvement of the ulcers (Figure 4).