Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Oct 26, 2022; 10(30): 10997-11003
Published online Oct 26, 2022. doi: 10.12998/wjcc.v10.i30.10997
Wells’ syndrome possibly caused by hematologic malignancy, influenza vaccination or ibrutinib: A case report
Mihela Šajn, Boštjan Luzar, Samo Zver
Mihela Šajn, Samo Zver, Department of Hematology, University Medical Centre Ljubljana, Ljubljana 1000, Slovenia
Boštjan Luzar, Institute of Pathology, Medical Faculty Ljubljana, Ljubljana 1000, Slovenia
Boštjan Luzar, Samo Zver, Medical Faculty, University of Ljubljana, Ljubljana 1000, Slovenia
Author contributions: Šajn M and Zver S designed the manuscript; Šajn M and Luzar B collected the patient’s clinical data; Šajn M, Luzar B and Zver S contributed to the analysis and interpretation of the data, and participated in drafting of the manuscript; Zver S critically revised the manuscript; all authors read and approved the final version of the manuscript.
Informed consent statement: Written informed consent was obtained from the patient before treatment.
Conflict-of-interest statement: All authors report no relevant conflict of interest for this article.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Mihela Šajn, MD, Doctor, Department of Hematology, University Medical Centre Ljubljana, Zaloška Cesta 7, Ljubljana 1000, Slovenia. mihela.sajn@kclj.si
Received: June 20, 2022
Peer-review started: June 20, 2022
First decision: August 1, 2022
Revised: August 11, 2022
Accepted: September 6, 2022
Article in press: September 6, 2022
Published online: October 26, 2022
Processing time: 122 Days and 11.1 Hours
Abstract
BACKGROUND

Wells’ syndrome (eosinophilic cellulitis) is an uncommon eosinophilic dermatosis of uncertain pathogenesis, characterized by clinical polymorphism and suggestive but nonspecific histopathologic traits. Its course is recurrent, and response to therapy is unpredictable. In a case in which the patient has a number of potential triggers for the manifestation of Wells’ syndrome skin rash, the treating physician must decide or must make an assumption in order to establish the most likely clinical scenario. This is important for the patient’s future treatment plans.

CASE SUMMARY

We describe the clinical case of a 46-year-old female with chronic lymphocytic leukemia who had already received treatment for several months with ibrutinib. She was diagnosed with Wells’ syndrome 10 d after an influenza vaccination containing thimerosal. Based on the literature, the patient was treated with a course of oral steroids. Resolution of clinical symptoms and rash were observed in response to the treatment. Ibrutinib was not discontinued.

CONCLUSION

The etiology of Wells’ syndrome remains unknown. Clinically, it resembles bacterial cellulitis. Lack of response to antibiotic treatment should lead the physician to consider a diagnosis of Wells’ syndrome. Treating the underlying condition is important and may lead to resolution of the syndrome. However, the most common and effective treatment to limit the course of the disease are systemic steroids.

Keywords: Wells’ syndrome; Chronic lymphocytic leukemia; Allogenic hematopoietic stem cell transplantation; Ibrutinib; Thimerosal-containing influenza vaccine; Clinical case; Case report

Core Tip: Our patient presented with pruritic rash all over her body. Based on the pathohistological features, a diagnosis of Wells’ syndrome (eosinophilic cellulitis) was established. We considered hematological malignancy, ibrutinib and influenza vaccine as possible triggers. The only new event and therefore most probable trigger for Wells’ syndrome was an influenza vaccination with a vaccine containing thimerosal. Clinically, this is a relatively rare case.