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Mycosis fungoides (MF) represents approximately 50% of primary cutaneous lymphomas [1], of which the erythrodermic ulcerated form of MF is rare [2]. No therapy has been established. We present the case of an erythrodermic ulcerated form of MF patient that was treated effectively and long-term with brentuximab vedotin (BV). A 57-year-old woman with MF, stage IIIA (T4, Nx, M0, B0), presenting with widespread erosive erythema and enlarged cervical, axillary and inguinal lymph nodes (LN), was referred to us because of resistance to treatment with topical corticosteroid, ultraviolet phototherapy and oral bexarotene at 150 mg/day, administered at a reduced dose due to concerns about potential side effects. She declined hematopoietic stem cell transplant. The patient was treated with vorinostat at 400 mg/day, but erosive lesions spread. In addition, she developed sepsis, fungemia, and pulmonary embolism. Despite initiation of mogamulizumab at 1 mg/kg/week because of the refractory clinical course, the eroded surface continued to enlarge (Figure 1a). After five cycles of mogamulizumab, a skin biopsy was performed on the abdominal plaque, revealing diffuse infiltration of lymphocytes with nuclear atypia in the upper dermis with epidermotropism (Figure 1b,c). Immunohistochemical studies demonstrated positivity for CD3 and CD4, with scattered large CD30+ cells (Figure 1d–f). Granzyme B (GrB)+ cells were also seen (Figure 1g). Ki-67 labeling index was 29.9% (Figure 1h). Similar infiltration was observed in other erosive lesions. We administered BV at 70% dose of the standard dose (1.8 mg/kg), every 3 weeks, based on disease progression. The erythematosquamous plaques and ulcerations gradually improved and largely resolved after eight infusions of BV (Figure 1i). The lymphadenopathy had improved and all complications were fully controlled. However, following 43 infusions of BV over 30 months, new ulcerated plaques developed and enlarged LN also increased again. A biopsy specimen from the erythematous plaque on the back showed a dense, large atypical lymphoid infiltrate in the epidermis and upper dermis. Immunohistochemical staining revealed features similar to previous findings, along with CD30 expression < 10%, except the absence of GrB+ cells. We ended administration of BV at 45 infusions after MF exacerbated, and romidepsin therapy was started at 14 mg/m2. Eight days later, cytomegalovirus antigenemia was detected. Despite initiation of ganciclovir therapy, she developed cytomegalovirus-mediated colon perforation on the 10th day and died 36 months after the initial visit due to sepsis. To our knowledge, only five cases of the erythrodermic ulcerated form of MF, including our case, have been reported [2-5]. All patients showed no circulating Sézary cells and demonstrated dermal lymphoid infiltration mainly comprised of CD4+ T-cells, whereas CD30+ cell expression varied. The mechanisms of erosions in MF remain unclear, while one report suggested involvement of activated lymphocytes producing T-cell intracellular antigen-1 and GrB [4]. In our case, GrB+ cells observed after five cycles of mogamulizumab in erosive lesions disappeared after the BV therapy, and she maintained a sustained response for 30 months. These findings suggest activated lymphocytes might be related to erosive MF lesions, though further investigation is needed. Regardless of CD30 expression frequency, BV may be effective against the erythrodermic ulcerated form of MF. The authors have nothing to report. Shin Morizane is an Editorial Board member of the Journal of Dermatology and a co-author of this article. To minimize bias, he was excluded from all editorial decision-making related to the acceptance of this article for publication. Except for this, the authors have no conflicts of interest to declare. The authors have nothing to report.