Progression of Cutaneous T-Cell Lymphoma After Dupilumab Journal of the American Academy of Dermatology

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• Dupilumab, an IL-4 receptor, has been used off-label for cutaneous T-cell lymphoma (CTCL) due to the similar Th2 cytokine profile of CTCL to atopic dermatitis. The authors present a series of 7 patients with either CTCL (stages IB–IIIB) with severe pruritus (n = 3) or clinically presumed atopic dermatitis (AD; n = 4) who were treated with dupilumab. Of the total, 6 patients responded initially with decreased erythema and pruritus; however, this was followed by worsening body surface area involvement in all patients as well as pruritus, lymphadenopathy, and systemic symptoms (n = 5, 3, and 3, respectively). All 4 patients with atypical AD were eventually diagnosed with CTCL, and the 3 patients with preexisting CTCL developed disease progression, which in 2 cases led to Sézary syndrome and death.

• The mechanism of action of dupilumab in CTCL is not fully understood. In these 7 cases, dupilumab led to long-term worsening and progression of CTCL and death in 2 cases. In atypical AD patients, such as those with new locations of involvement, onset in elderly years, or lymphadenopathy, investigation for CTCL should be undertaken and dupilumab used with extreme caution.

– Margaret Hammond, MD

Written by

 

 

Eliot N Mostow MD, MPH 

Written by

 

 

Robert T BrodellMD, FAAD 

Written by

 

 

Ashish CBhatia MD, FAAD

This article is important because it plays into the importance of evidence-based treatment following an accurate diagnosis. In a sincere attempt to help people with our new targeted biologic therapies, dermatologists can fall into a trap by using powerful treatments on new conditions believing they could do little harm while recognizing there are inadequate data. In this cautionary tale, treating patients with CTCL appears to lead to short-term gains, followed by more rapid progression of disease than would normally be expected. Similarly, patients with presumed atopic dermatitis who later transformed to or were eventually diagnosed with CTCL showed similar disease progression. This could certainly occur in any of our practices.

This is a small series, and it's hard to know for certain if a larger series would reproduce these findings, but it reminds me to "shoot from the hip" with necessary humility and caution. This is not to say that we shouldn't try things based on limited experience and good intentions; however, we should be certain that the patient understands our efforts are based on limited knowledge and understanding traditional approaches, and provides informed consent.

Abstract

Dupilumab is a fully human monoclonal antibody that binds the interleukin-4 receptor alpha (IL-4R) subunit and inhibits signaling of IL-4 and IL-13. It was approved by the Food and Drug Administration for the treatment of moderate-to-severe atopic dermatitis (AD) in adults and adolescents with efficacy in reducing pruritus.1 The two most common subtypes of cutaneous T-cell lymphoma (CTCL) are mycosis fungoides (MF) and Sézary syndrome (SS). MF/SS and AD are similarly driven by T-helper 2 (TH2) cytokine profiles and may present with similar morphology (i.e. erythema, lichenification, fissuring) with pruritus, disruption of the skin barrier, and impetiginization. Thus, it has been hypothesized that dupilumab may be effective in treating CTCL.

Journal of the American Academy of Dermatology

Progression of Cutaneous T-Cell Lymphoma After Dupilumab: Case Review of 7 Patients

J Am Acad Dermatol 2020 Mar 27;[EPub Ahead of Print], ML Espinosa, MT Nguyen, AS Aguirre, ME Martinez-Escala, J Kim, CJ Walker, DS Pontes, JI Silverberg, J Choi, B Pro, LB Pincus, J Guitart, XA Zhou 

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Benjamin Hidalgo-Matlock
Skin Care Physicians of Costa Rica

Clinica Victoria en San Pedro: 4000-1054
Momentum Escazu: 2101-9574

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