Pyoderma vegetans (PV) or blastomycosis-like pyoderma (BLP) is a chronic inflammatory disease, by some authors considered a rare variety of pyoderma gangrenosum (PG), and others describe it as a distinct entity. It commonly presents with verrucous plaques with multiple pustules. The etiology of this disease is unknown, but it has been connected with staphylococcal and streptococcal infections, inflammatory bowel disease, hematological diseases, primary immunodeficiency, alcoholism, and nutritional deficit. Here we present a 66-year-old, otherwise healthy female, with a 2-year-long history of well-defined, vegetative livid plaques with multiple pustules on the dorsal side of both hands. Histopathological analysis of the skin biopsy of the hand showed chronic inflammation and micro-abscesses, ruptured follicular cysts and follicular pseudoepitheliomatous hyperplasia. Treatment with anti-tuberculosis drugs and antibiotics showed to be ineffective, as well as the treatment with systemic corticosteroids, dapsone and cyclosporine. Itraconazole was given for its immunomodulatory effects and findings of Penicillium species in one of the swabs, which led to partial regression of lesions. Since the treatment did not lead to complete resolution, acitretin was indicated 3 months later, but the patient was lost to follow-up.