Development of Sézary syndrome following the administration of dupilumab

Tran, Jessica; Morris, Lisa; Vu, Alan; Duvic, Madeleine

doi:10.5070/d3264048364

Cited by 24 publications

(31 citation statements)

References 10 publications

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“…However, 7 of those experienced disease progression with two reported deaths; an average length of initial improvement of 1.9 months was reported for 6 patients. Other reports of 8 patients showed no response, primary worsening or new development of CTCL under dupilumab [ 274 , 275 , 276 , 277 , 278 , 279 , 280 , 281 , 282 ]. Possible mechanisms could be increased binding of IL-13 to IL-13RA2 expressed on lymphoma cells that is not inhibited by dupilumab.…”

Section: Resultsmentioning

confidence: 98%

Dupilumab in Inflammatory Skin Diseases: A Systematic Review

et al. 2023

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Dupilumab was first approved for the treatment of atopic dermatitis (AD) and blocks the signaling of interleukin (IL)-4 and -13. Several other chronic skin conditions share mechanistic overlaps with AD in their pathophysiology, i.e., are linked to type 2 inflammation. Most recently, dupilumab was approved by the U.S. Food and Drug Administration for prurigo nodularis (PN). Given its relatively good safety profile, effective off-label use of dupilumab has been reported for a multitude of dermatologic diseases and several clinical trials for dermatologic skin conditions are currently ongoing. We conducted a systematic review of applications of dupilumab in dermatology other than AD and PN by searching the databases PubMed/Medline, Scopus, Web of Science and Cochrane Library as well as the clinical trial registry ClinicalTrials.gov. We found several reports for effective treatment of bullous autoimmune diseases, eczema, prurigo, alopecia areata, chronic spontaneous urticaria, Netherton syndrome and a variety of other chronic inflammatory skin diseases.

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Section: Resultsmentioning

confidence: 98%

Dupilumab in Inflammatory Skin Diseases: A Systematic Review

et al. 2023

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“…In recent years, several case studies have associated the unmasking or progression of CTCL with dupilumab treatment [ 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 ]. Dupilumab competitively inhibits IL-4 and IL-13 at the IL-4 α1 subunit receptor, preventing the downstream activation of tyrosine kinases promoting gene-transcription and related to both barrier dysfunction and Th2-mediated inflammation [ 15 ].…”

Section: Discussionmentioning

confidence: 99%

“…Whether these prior biopsies were undiagnosed “early” CTCL, or the dupilumab did in fact induce or exacerbate CTCL, is an area for further exploration. Of the 14 cases that include the CD4:CD8 ratio, all had a greater proportion of CD4 cells than CD8 cells [ 6 , 8 , 11 , 12 , 13 ]. Eight of these studies showed a CD4:CD8 ratio of ≥20:1 [ 6 , 11 , 12 ].…”

Section: Discussionmentioning

confidence: 99%

“…Of the 14 cases that include the CD4:CD8 ratio, all had a greater proportion of CD4 cells than CD8 cells [ 6 , 8 , 11 , 12 , 13 ]. Eight of these studies showed a CD4:CD8 ratio of ≥20:1 [ 6 , 11 , 12 ].…”

Section: Discussionmentioning

confidence: 99%

“…In 2017, dupilumab was approved by the United States Food and Drug Administration (FDA) for the treatment of moderate-to-severe AD in adults uncontrolled with topical medications, becoming the first biologic agent available to treat AD [ 1 ]. Several case studies in recent years have associated unmasking or progression of cutaneous T-cell lymphomas (CTCL) with dupilumab treatment [ 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 ]. To date, all cases that have reported a CD4:CD8 ratio have shown a greater proportion of CD4+ cells [ 14 ].…”

Section: Introductionmentioning

confidence: 99%

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Dupilumab-Associated Mycosis Fungoides with a CD8+ Immunophenotype

et al. 2022

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Dupilumab is a humanized IgG4 monoclonal-antibody that is approved by the United States Food and Drug Administration (FDA) for the treatment of moderate-to-severe atopic dermatitis (AD) in patients aged 12 years and older. In recent years, several case studies have associated the unmasking or progression of cutaneous T-cell lymphomas (CTCL) with dupilumab treatment. To date, all reported cases of dupilumab-associated CTCL have shown a CD4+ T-helper-cell-predominant immunophenotype. Here, we report a case of a 72-year-old man who presented with a 2-year history of a diffuse, pruritic eruption, who was started on dupilumab for 9 weeks. He subsequently developed mycosis fungoides (MF) with a CD8+-predominant immunophenotype. Overall, cases of CD8+ mycosis fungoides are less common and relatively less understood than their CD4+ counterparts, with varied presentations and courses. We present a case of dupilumab-associated CD8+ MF to highlight this presentation for pathologists and providers.

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