Dupilumab in eosinophilic cellulitis (Wells’ syndrome) – case report of a potential new treatment option

Traidl, Stephan; Angela, Yenny; Kapp, Alexander; Suhling, Hendrik; Schacht, Vivien; Werfel, Thomas

doi:10.1111/ddg.14598

Cited by 10 publications

(12 citation statements)

References 17 publications

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“…The overall recalcitrant nature of this lesion led to the innovative use of dupilumab combined with oral corticosteroids which resulted in complete resolution. This particular treatment regimen proved to be clinically efficacious similar to a case recently described by Traidl et al 6 One distinction between our case and that described by Traidl et al was that their patient had an additional history of severe eosinophilic asthma and nasal polyps and was previously being treated with benralizumab (IL-5 receptor antibody). 6 Furthermore, the patient interestingly had a relapse of their skin lesions 1 month into initiating dupilumab, however the patient's condition ultimately resolved after 6 months of dupilumab therapy.…”

Section: Discussionsupporting

confidence: 83%

“…This particular treatment regimen proved to be clinically efficacious similar to a case recently described by Traidl et al 6 One distinction between our case and that described by Traidl et al was that their patient had an additional history of severe eosinophilic asthma and nasal polyps and was previously being treated with benralizumab (IL-5 receptor antibody). 6 Furthermore, the patient interestingly had a relapse of their skin lesions 1 month into initiating dupilumab, however the patient's condition ultimately resolved after 6 months of dupilumab therapy. These shared observations warrant future consideration of the role that IL-4 and IL-13 receptor inhibition may play in the treatment of eosinophilic cellulitis, as it is postulated that dupilumab may function by modulating eosinophil trafficking to the dermis or by inhibiting the release of IL-5 through an IL-4 mediated mechanism within eosinophils.…”

Section: Discussionsupporting

confidence: 83%

“…These shared observations warrant future consideration of the role that IL-4 and IL-13 receptor inhibition may play in the treatment of eosinophilic cellulitis, as it is postulated that dupilumab may function by modulating eosinophil trafficking to the dermis or by inhibiting the release of IL-5 through an IL-4 mediated mechanism within eosinophils. 6,7 In conclusion, for refractory cases of Wells syndrome that are not responsive to first-line therapies, dupilumab may function as a potential treatment option.…”

Section: Discussionmentioning

confidence: 99%

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Atypical Wells syndrome successfully treated with dupilumab

McMullan

Torre

Santiago

et al. 2023

Skin Health and Disease

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Wells syndrome, also referred to as eosinophilic cellulitis, is a rare and often sporadic inflammatory skin condition whose aetiology remains uncertain. Clinically, this condition presents as a collection of erythematous, oedematous, and tender skin lesions most often affecting the extremities and trunk that can mimic cellulitis. Histologically, Wells syndrome is characterised by inflammatory changes and eosinophilic infiltration of the dermis with the absence of underlying infection, thereby distinguishing it from cellulitis. Due to the rarity of this syndrome and its ambiguous presentation, there remains to be a definitive strategy for treatment. Recent case reports have documented varying success and recurrence with the use of oral and topical corticosteroids, antifungals, antibiotics, immunosuppressants and antihistamines. Here, we report a unique case of progressively worsening neutrophilic‐rich Wells syndrome on the vertex of the scalp that was successfully treated with a combination of dupilumab and oral corticosteroids.

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Section: Discussionsupporting

confidence: 83%

Section: Discussionsupporting

confidence: 83%

Section: Discussionmentioning

confidence: 99%

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Atypical Wells syndrome successfully treated with dupilumab

McMullan

Torre

Santiago

et al. 2023

Skin Health and Disease

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“…Four cases of steroid-refractory papuloerythroderma Ofuji treated with dupilumab all reported complete resolution of skin lesions and pruritus [ 236 , 237 , 238 ]. Two cases of highly steroid- and immunosuppressant-refractory Well’s syndrome reported complete resolutions [ 239 , 240 ], as did two cases of the related erythema annulare eosinophilicum [ 241 , 242 ]. One case of eosinophilic fasciitis reported resolution of skin induration clinically and in MRI-studies under dupilumab treatment [ 243 ].…”

Section: Resultsmentioning

confidence: 99%

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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“…2 After an extensive literature search, several case studies reporting successful treatment with biologics came to our attention. [3][4][5][6][7] Considering the patient's history of EGPA and the widespread skin lesions, we started therapy with mepolizumab, a monoclonal antibody that targets interleukin-5 (IL-5). Subcutaneous injections with 100 mg mepolizumab were administered once every 4 weeks.…”

Section: Histological Evaluation Showed Edema Eosinophilic Infiltrate...mentioning

confidence: 99%