Vasculitic urticaria as a sign of malignancy

Abstract

Adult T-cell leukemia/lymphoma is a rare complication of human T-lymphotropic virus type 1 (HTLV-1) infection. It is a diagnostic challenge due to its many systemic manifestations, with skin involvement being common.

We present the case of a 72-year-old woman with a three-month history of urticarial-like lesions refractory to steroid and antihistamine treatment, along with a one-year history of dyspnea. A skin biopsy showed vasculitic urticaria, and the chest tomography findings were highly suggestive of lymphangitic carcinomatosis. A transbronchial biopsy reported a highly undifferentiated neoplasm. In a search for the primary tumor, she underwent a biopsy of gastric and colon polyps, with an anatomopathological report of adult T-cell lymphoma vs. nonspecific T-cell lymphoma. Both ELISA and PCR tests for HTLV-1 were therefore ordered, confirming viral infection. She received first-line treatment with chemotherapy which was discontinued due to pulmonary toxicity. This was followed by second line treatment with antiviral therapy using lamivudine + interferon alpha-2b, which was stopped three months later due to bone marrow failure. She finally died from urinary septic shock 11 months after the initial diagnosis. The skin manifestations and anatomopathological findings of adult T-cell lymphoma are diverse, polymorphic and associated with a poor prognosis.

Our case is, so far, the only one with documented findings of vasculitic urticaria as a paraneoplastic manifestation. The combination of histopathological, laboratory (serum HTLV-1 identification) and clinical findings is essential for its diagnosis. It is scientifically relevant because it is an underdiagnosed disease with a very high mortality. Therefore, more research is needed on the prevention of complications and search for treatment to improve survival.