Introduction. Pityriasis lichenoides et varioliformis acuta is a rare inflammatory skin disease of unknown etiology and its diagnosis is sometimes established by eliminating diseases that are considered in the differential diagnosis. Given the lack of randomized clinical trials, recommendations for therapy remain based on case reports and case series. Case Report. We present a 63-year-old female patient with generalized skin lesions including, papules, papulonecrotic lesions, and atrophic scars accompanied by a subjective feeling of itching that occurred 2 months before admission. The histopathological findings showed a mixed perivascular inflammatory cellular infiltrate and capillary blood vessels with thickened walls in the superficial part of the dermis as signs of vasculitis. The infiltrate was dominated by lymphocytes, neutrophils were admixed, but there were no signs of cellular atypia, which supported the clinical diagnosis of pityriasis lichenoides et varioliformis acuta. Therapy with systemic corticosteroids and doxycycline was applied, which led to the resolution of lesions. Conclusion. The authors would like to bring to the readers? attention a rare skin disease, pityriasis lichenoides et varioliformis acuta, point to papulonecrotic tuberculids in differential diagnosis due to similar clinical presentation, remind them of the dilemmas that may arise in case of the described lymphocytic vasculitis based on the findings of histopathological analysis, and highlight the effectiveness of doxycycline and prednisone in the therapy.