Dupilumab therapy in Sézary syndrome misdiagnosed as atopic dermatitis: A case report

Umemoto, Naoka; Demitsu, Toshio; Otaki, Kaoru; Matsumoto, Takanao; Takazawa, Maya; Yamada, Atsushi; Kimura, Shun‐ichi; Kakurai, Maki

doi:10.1111/1346-8138.15501

Cited by 17 publications

(33 citation statements)

References 5 publications

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“…At the same time, we observed transient increase in peripheral Sézary cell counts. This raises safety concerns, in particular in the light of recent reports of worsening CTCL cases under dupilumab 12–14 . However, our case is distinct from previously reported cases in several aspects.…”

Section: Discussioncontrasting

confidence: 57%

“…In previously reported cases, dupilumab was given without flanking anti-tumour therapy to patients who were treated for misdiagnosed AD. 13,14 Further, dupilumab seems better suited for the treatment of SS than MF, since MF is not an equally Th2-biased disease and leukaemic MF represents an immunologically different lymphoma than SS. 8,12 Thus, the differences seen between our patient and published cases might result from differences in the biology of the underlying malignancy, the flanking anti-cancer therapy and the stage of tumour control at initiation of dupilumab treatment.…”

Section: Discussionmentioning

confidence: 99%

“…In contrast to reported cases, we used dupilumab as supportive treatment in conjunction with continued ECP. In previously reported cases, dupilumab was given without flanking anti‐tumour therapy to patients who were treated for misdiagnosed AD 13,14 . Further, dupilumab seems better suited for the treatment of SS than MF, since MF is not an equally Th2‐biased disease and leukaemic MF represents an immunologically different lymphoma than SS 8,12 .…”

Section: Discussionmentioning

confidence: 99%

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Rapid and sustained control of itch and reduction in Th2 bias by dupilumab in a patient with Sézary syndrome

Steck

Bertschi

Luther

et al. 2020

Acad Dermatol Venereol

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Background S ezary syndrome is a leukaemic variant of cutaneous T-cell lymphoma with poor prognosis. With the exception of stem cell transplantation, current treatments for SS are not curative. Rather, they aim at reducing disease burden and improving quality of life. Yet, pruritusthe major cause for impaired quality of life in these patientsis notoriously difficult to treat. Thus, supportive treatments addressing agonizing pruritus are urgently needed.Objectives To explore the clinical and immunological effects of type 2 cytokine blockade with dupilumab as supportive treatment in S ezary syndrome.Methods A S ezary syndrome patient with stable disease but intractable pruritus was treated with dupilumab in combination with continued extracorporeal photopheresis. Close clinical and immunological monitoring on blood and skin samples from the patient was performed over 44 weeks. In vitro assays with patient's lymphoma cells were performed to address effects of dupilumab on S ezary cell's response to Th2 cytokines.Results Clinically, dupilumab treatment induced rapid and sustained reduction in itch and improvement of skin and lymph node involvement. In both blood and skin, a reduction in Th2 bias was observed. Intriguingly, lymphocyte counts and S ezary cells in blood increased and later stabilized under dupilumab treatment. In vitro, dupilumab abrogated the anti-apoptotic and activating effects of Th2 cytokines on S ezary cells.Conclusions In this S ezary patient, inhibition of IL-4 and IL-13 signalling was associated with striking clinical benefit in terms of quality of life, pruritus and use of topical corticosteroids. While safety remains an important concern, our data support the future exploration of Th2 modulation for supportive care in S ezary Syndrome.

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Section: Discussioncontrasting

confidence: 57%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Rapid and sustained control of itch and reduction in Th2 bias by dupilumab in a patient with Sézary syndrome

Steck

Bertschi

Luther

et al. 2020

Acad Dermatol Venereol

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“…Indeed, administration of Toll-like receptor (TLR) agonists boosting cellular immunity has shown clinical efficacy, and treatment with IL-12 and IFNγ can induce regression of CTCL lesions which is associated with increased numbers of CD8 T cells in the resolving skin ( Rook et al, 1999 , 2001 , 2015 ; Suchin et al, 2002 ; Dummer et al, 2004 ; Duvic et al, 2006 ; Wysocka et al, 2007 ; Kim et al, 2010 ; Accart et al, 2013 ). Of notice, recent case reports have surprisingly described that long-term treatment with dupilumab, a neutralizing antibody targeting IL-4 receptor alpha, may exacerbate CTCL and possibly even trigger the disease in certain patients with severe atopic dermatitis (AD) ( Chiba et al, 2019 ; Espinosa et al, 2020 ; Miyashiro et al, 2020 ; Tran et al, 2020 ; Umemoto et al, 2020 ). While substantial data currently support that the transition from a Th1- to a Th2-biased tumor microenvironment contributes to the progression of CTCL, these new findings suggest that the role of the cytokine milieu might be more complex than appreciated thus far.…”

Section: Th2-bias During Disease Progressionmentioning

confidence: 99%

Cellular Interactions and Inflammation in the Pathogenesis of Cutaneous T-Cell Lymphoma

Stolearenco

Namini

Hasselager

et al. 2020

Front. Cell Dev. Biol.

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Cutaneous T-cell lymphoma (CTCL) comprises a group of lymphoproliferative diseases characterized by the accumulation of malignant T cells in chronically inflamed skin lesions. In early stages, the disease presents as skin patches or plaques covering a limited area of the skin and normally follows an indolent course. However, in a subset of patients the cutaneous lesions develop into tumors and the malignant T cells may spread to the lymphatic system, blood and internal organs with fatal consequences. Despite intensive research, the mechanisms driving disease progression remain incompletely understood. While most studies have focused on cancer cell-intrinsic oncogenesis, such as genetic and epigenetic events driving malignant transformation and disease progression, an increasing body of evidence shows that the interplay between malignant T cells and non-malignant cells plays a crucial role. Here, we outline some of the emerging mechanisms by which tumor, stromal and epidermal interactions may contribute to the progression of CTCL with particular emphasis on the crosstalk between fibroblasts, keratinocytes and malignant T cells.

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“…Contrary to these results, one case reported a patient diagnosed with both MF and AD had a good clinical outcome, with improvements noticed in both conditions [ 37 ]. There have also been reports of patients diagnosed with AD that developed SS following Dupilumab administration [ 37 , 38 ]. No improvement in skin condition was noted in these cases, whereas another case describing Dupilumab administration for a patient diagnosed from the start with SS noted a great improvement in skin disease, pruritus, and quality of life [ 39 ].…”

Section: Discussionmentioning

confidence: 99%

Atopic Dermatitis with Multiple Comorbidities Treated with Dupilumab. A Case Report and Review of the Literature Regarding the Safety of Dupilumab

Mitroi

Stoica

Mitroi

et al. 2022

Life

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Dupilumab is the only available biological treatment for moderate-to-severe atopic dermatitis (AD). Even so, limited clinical data regarding its safety profile are available. Interactions with other drugs and the adverse effects of Dupilumab on patients with multiple comorbidities, such as chronic heart disease, diabetes, chronic kidney disease, etc., are not known yet. Moreover, there have been described cases of cutaneous lymphomas induced by Dupilumab. Therefore, the clinician that wants to start treatment for moderate-to-severe atopic dermatitis, which does not respond to conventional drugs, might be reluctant to choose biologic agents such as Dupilumab. In this paper, we reported a case of severe atopic dermatitis with multiple comorbidities in which the patient was successfully treated with Dupilumab despite numerous underlying conditions. We also conducted a review of the current literature on the safety profile of Dupilumab in special categories of patients with comorbidities, such as heart, kidney, and liver disease, oncologic conditions, and during pregnancy.

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Dupilumab therapy in Sézary syndrome misdiagnosed as atopic dermatitis: A case report

Cited by 17 publications

References 5 publications

Rapid and sustained control of itch and reduction in Th2 bias by dupilumab in a patient with Sézary syndrome

Rapid and sustained control of itch and reduction in Th2 bias by dupilumab in a patient with Sézary syndrome

Cellular Interactions and Inflammation in the Pathogenesis of Cutaneous T-Cell Lymphoma

Atopic Dermatitis with Multiple Comorbidities Treated with Dupilumab. A Case Report and Review of the Literature Regarding the Safety of Dupilumab

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