Dupilumab
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Sezary syndrome: case report A 64-year-old man developed Sezary syndrome during treatment with dupilumab for atopic dermatitis. The man, who had diagnosed with atopic dermatitis in 2014, was admitted to MD Anderson Cancer Center in September 2019 with a 3 months history of erythroderma. He had a history of taking UV light therapy and topical corticosteroids for atopic dermatitis. In June 2019, he was administered a loading dose of SC dupilumab 600mg. Two weeks later, erythrodermic rash which covers 95% of his body surface was observed. Methylprednisolone treatment was given and the man’s symptoms started resolving, but relapsed easily as the dose were tapered. He subsequently received 4 injections of dupilumab leading to progression of the erythroderma. His pathology reports revealed superficial perivascular lymphohistiocytic infiltrate, hypogranulosis, neutrophils, and psoriasiform epidermal hyperplasia within the epidermis. There was also presence of lymphocytic exocytosis with some small intraepidermal lymphocytes that showed convoluted nuclear contours. He was diagnosed with psoriasis. His treatment initiated with ciclosporin for 2 months, guselkumab for 1 month subsequently with topical steroids, but his lymphadenopathy and erythroderma persisted. In September 2019, results from flow cytometry revealed increased in the ration of CD4 to CD8. It also showed increased in the level of atypical CD4+ Tlymphocytes which leads to loss of CD7 expression. Gene rearrangement studies of T-cell receptor showed rearrangements of polyclonal TCR-beta and monoclonal TCR-gamma gene in both skin and blood. These findings lead to identification of Sezary syndrome. He was again referred to MD Anderson Cancer Center presented with erythema, mild pruritus, exfoliation, and a severe burning sensation. Physical investigation showed plantar surfaces and hyperkeratotic palmar, mild bilateral ectropion, and scaling of bilateral toes. Sezary syndrome was confirmed. Treatment with biweekly extracorporeal photopheresis, and oral bexarotene was initiated. Additionally, topical desoximetasone was also applied to the affected area [outcome not stated]. Tran J, et al. Development of Sezary syndrome following the administration of dupilumab. Dermatology Online Journal 26: No. 4, Apr 2020. Available from: URL: https:// 803497623 escholarship.org/uc/item/1m67z8sb
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Reactions 22 Aug 2020 No. 1818
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