Wells Syndrome with Sustained Response to Omalizumab

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Kezia Surjanto
Gage Rensch
Garrett Vick
Andrea Murina

Keywords

Wells syndrome, eosinophilic cellulitis, flame figure, omalizumab

Abstract

Wells syndrome (eosinophilic cellulitis), a rare dermatosis that arises from a multitude of triggers, may present with pruritic and erythematous plaques, papules, vesicles, or blisters.1 We present a case of Wells syndrome that was successfully treated with omalizumab. A 39-year-old female presented with a three-year history of diffusely located pruritic papules. After a negative lab and infectious workup, a punch biopsy showed dense eosinophilic infiltrate in the dermis as well as “flame figure” formation. Unsuccessful trials of prednisone, cetirizine, hydroxyzine, and dapsone prompted the use of omalizumab, which yielded excellent control of symptoms. Omalizumab’s success in the Wells syndrome disease process may be explained by its anti-inflammatory effects on IgE autoantibodies and receptors.5 Few cases of Wells syndrome treated with omalizumab have been reported. Thus, increased research to thoroughly elucidate omalizumab’s pharmacological mechanism of action in this particular disease process is warranted and would be a valuable contribution to the Wells syndrome literature.

References

1. Weins AB, Biedermann T, Weiss T, Weiss JM. Wells syndrome. JDDG: Journal der Deutschen Dermatologischen Gesellschaft. 2016;14(10):989-993.

2. Egeland Ø, Balieva F, Undersrud E. Wells syndrome: a case of successful treatment with omalizumab. Int J Dermatol. 2018;57(8):994-995.

3. Ogueta I, Spertino J, Deza G, et al. Wells syndrome and chronic spontaneous urticaria: report of four cases successfully treated with omalizumab. J Eur Acad Dermatol Venereol. 2019;33(10).

4. Caputo R, Marzano AV, Vezzoli P, Lunardon L. Wells syndrome in adults and children: a report of 19 cases. Arch Dermatol. 2006;142(9).

5. Kaplan AP, Giménez-Arnau AM, Saini SS. Mechanisms of action that contribute to efficacy of omalizumab in chronic spontaneous urticaria. Allergy. 2017;72(4):519-533.

6. Coattrenec Y, Ibrahim Yasmine L, Harr T, Spoerl D, Jandus P. Long-term remission of wells syndrome with omalizumab. J Investig Allergol Clin Immunol. 2020;30(1):58-59.

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